Hematology

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Hematology
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2011-05-21 22:20:32
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DPAP2012 Hematology
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Hematology cards made by previous students
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  1. Hypoproliferative Anemia
    90% production problem (bone marrow fails to respond to anemia)
  2. Hyperproliferative Anemia
    bone marrow is not able to compensate for blood loss or destruction by ramping up RBC production
  3. Microcytic Anemia
    • decreased RBC production;
    • severe due to either iron deficiency or thalassemia
    • (also chronic inflammation or sideroblastic)
  4. Normocytic Anemia
    decreased RBC production
  5. Macrocytic Anemia
    • decreased RBC production;
    • severe is either B12 or folate deficiency or occ MDS
  6. Thalassemia
    hereditary disorders which affect the production of globin chains (alpha or beta)
  7. Alpha Thalassemia
    due to gene deletion causing a reduction in alpha chain synthesis (severity depends on how many deletions)
  8. Beta Thalassemia
    due to gene mutations which cause RBC membrane damage which can lead to hemolysis
  9. Iron Deficiency Anemia
    most common cause of anemia; diet (foods, caloric intake, absorptive capacity, increased loss*, increased requirements)
  10. Anemia of Chronic Disease
    • liver disease,
    • acute or chronic infection (HIV),
    • chronic inflammation,
    • hypothyroidism,
    • renal disease (hypertensive/diabetic),
    • cancer (could be result of reduced erythropoietin stimulation)
  11. Sideroblastic Anemia
    • Enzyme disorder in which body has adequate iron, but is unable to incorporate it into Hgb (iron accumulates in mitochondria of RBC);
    • can be inherited, acquired or idiopathic
  12. B12 Deficiency
    • B12 must come from diet (2000-5000mcg of stored B12 in liver so takes 3+ yrs to become deficient);
    • inadequate intake,
    • malabsorption* (intrinsic factor, gut or Rx);
    • pernicious (familial)
  13. Folate Deficiency
    • total body stores are 5-20mg so deficiency can occur in a matter of months;
    • inadequate intake*,
    • increased requirements,
    • malabsorption,
    • impaired metabolism;
    • Rx interactions
  14. Pure Red Cell Aplasia
    • rare;
    • either idiopathic or acquired;
    • autoimmune disease mediated by T cells or rarely IgG antibody;
    • thymoma, lymphoid malignancies, solid tumors, SLE, RA, infections, medications
  15. Aplastic Anemia
    • bone marrow failure bc of injury or suppression of hematopoietic stem cell;
    • acquired (Rx, chemo, radiotherapy, chemicals, viruses, preggers, lupus, gvhd);
    • hereditary rare;
    • idiopathic*
  16. Hemolytic Anemias
    • RBCs are destroyed either episodically or continuously;
    • anemia results when bone marrow is not able to keep up with rate of destruction;
    • cause of destruction is either due to an intrinsic defect or some external factor
  17. Hereditary Spherocytosis
    inherited abnormality of RBC membrane (molecular defect in spectrin protein)
  18. Paroxysmal Nocturnal Hemoglobinuria
    acquired stem cell disorder making RBC membrane prone to lysis by complement (due to lack of special proteins)
  19. Glucose 6 Phosphate Dehydrogenase Deficiency
    • hereditary enzyme defect causing episodic hemolytic anemia related to oxidative stress;
    • X linked recessive (200 million affected worldwide)
  20. Sickle Cell Disorders
    • Hgb S; autosomal recessive;
    • onset in first year of life when Hgb F levels fall;
    • avg life expectancy 40-50 yrs;
    • trait have no anemia
  21. Autoimmune Hemolytic Anemia
    • acquired disorder which IgG autoantibody formed that binds to RBC membrane and causes destruction in spleen and liver;
    • 50% idiopathic;
    • occurs at all ages
  22. Cold Agglutinin Disease
    • acquired hemolytic anemia due to IgM autoantibody;
    • only reacts at temperature lower than 37degreesC;
    • IgM binds with complement which causes destruction of RBC within liver;
    • most idiopathic
  23. Neutropenia
    bone marrow disorder or peripheral disorders
  24. Polycythemia Vera (PCV)
    • myeloproliferative disorder;
    • increased RBCs resulting from a clonal multipotent hematopoietic stem cell defect;
    • primary: true increase,
    • secondary: increase due to another condition such as hypoxia, renal disease, MI's, dehydration..
  25. Thrombocytosis
    • increased sensitivities to cytokines which stimulate bone marrow cell proliferation and growth;
    • decreased inhibition of plt inhibiting factors;
    • defects in cellular microenvironment;
    • JAK-2 mutations
  26. Myelofibrosis
    • cause unknown;
    • bone marrow is replaced with scar tissue, leading to anemia and eventually marrow failure;
    • an abnormal myeloid precursor is believed to give rise to dysplastic megakaryocytes that produce increased fibroblast factors
  27. Myelodysplastic Syndrome
    • acquired clonal disorders of the hematopoietic stem cell;
    • affect one or more cell lines;
    • occurs b/c blood cells do not develop into mature cells so they are not released into blood and accumulation of immature cells occur in bone marrow
  28. Multiple Myeloma
    • etiology unknown; plasma cells crowd out other cells in bone marrow;
    • collection in bone marrow = myeloma,
    • multiple collections seen as multiple bone lesions = multiple myeloma
  29. Monoclonal Gammopathy of Unknown Significance (MGUS)
    • IgG spike;
    • M spike stable
  30. Waldenström’s Macroglobulinemia
    • malignancy of B cells;
    • causes overproduction of monoclonal macroglobulin (IgM)
  31. Chronic Lymphocytic Leukemia
    • most common leukemia;
    • clonal proliferation and accumulation of mature-appearing B cells in blood and lymphoid tissue
  32. Chronic Myelogenous Leukemia
    • too many WBCs/granulocytic cells made in bone marrow (esp myeloid cells);
    • 3 phases defined by the # of blasts in marrow (chronic, accelerated, acute);
    • blast crisis when blasts compromise >30% of bone marrow cells;
    • accounts for 7-20% of leukemia
  33. Acute Lymphoblastic Leukemia
    • neoplasm of immature lymphoblasts w markers of B or T cell lineage;
    • bone marrow (>20%) and peripheral blood (>10%) and frequently in the liver, spleen, lymph nodes and other organs;
    • common in children
  34. Acute Myelogenous Leukemia
    can be associated with exposure to toxins (benzenes, radiation, chemo)
  35. Hodgkin's Lymphoma
    usually arises in single area and spreads to contiguous
  36. Non-Hodgkin's Lymphoma
    • 5th most common malignancy in the US;
    • arise from cells residing in lymphoid tissue (90% of cases arise from B cells);
    • incidence higher in pts with immunodeficiencies
  37. Infectious Mononucleosis
    EBV
  38. Ehrlichiosis
    tick-borne Ehrlichiae bacteria
  39. Histoplasmosis
    intracellular fungus Histoplasma capsulatum
  40. Acute Thrombocytopenia
    decrease in plt count due to: decreased production (bone marrow), increased destruction (meds, immune mediated), sequestration (splenomegaly)
  41. Idiopathic (Immune) Thrombocytopenic Purpura
    • antibodies form and destroy plts (underlying illness: lupus, lymphoma, etc);
    • increased bone marrow production but cannot keep up with destruction
  42. Heparin Induced Thrombocytopenia
    transient decrease in plt count (type I) or immune mediated (type II, is subset of ITP)- dev'p antibodies to FIV
  43. Thrombotic Thrombocytopenic Purpura
    • disorder of von Willebrand Factor processing;
    • causes intra-vascular plt aggregation;
    • leads to systemic manifestations (CNS, renal, cardiac, hematologic)
  44. Hemolytic Uremic Syndrome
    • related to TTP;
    • associated with infectious diarrhea;
    • RBCs destroyed and kidneys stop function
  45. Coagulation: prolonged PT
    • deficiency in FVII and common pathway factors (FI, FII, FV, FX);
    • caused by liver disease, warfarin therapy, vitamin K deficiency, inhibitor to FVIIa
  46. Liver Disease (prolonged PT)
    liver is where you make all of your factors except for FVIII
  47. Vitamin K deficiency (prolonged PT)
    • have to have vitamin K in liver to make clotting factors (esp II, VII, IX, X) and Protein C and S (these are naturally occuring anticoagulants in body);
    • causes of deficiency: biliary tract disease, Rx, malnutrition
  48. Coagulation: prolonged aPTT
    deficiency in intrinsic (HMWK, PK, FXII, FXI, FIX. FVIII) and common pathway factors; caused by heparin and DTI therapy, inhibitors to coagulation factors
  49. von Willebrand Disease
    • most common hereditary bleeding disorder (1%);
    • mucocutaneous bleeding;
    • deficient in vWF quantity or quality (defect in plt adhesion);
    • have enough plts but not functioning properly
  50. Glanzmanns thrombasthenia
    • rare autosomal recessive disorder;
    • have enough plts but not functioning properly;
    • plts unable to aggregate due to defect in IIb/IIIa receptors
  51. Bernard-Soulier
    have enough plts but not functioning properly; reduced or abnormal plt vWF receptor
  52. Acquired Thrombopathy
    • have enough plts but not functioning properly;
    • drugs ASA, NSAIDS, cephalosporins;
    • renal disease;
    • alcohol
    • end-stage liver disease
  53. Prothrombin 20210A Mutation
    • elevated levels of prothrombin;
    • leads to a modest increase in hyper-coaguability;
    • second most common inherited cause of hypercoaguability
  54. anticoagulation disorders
    • Protein C or S deficiency;
    • anti-phospholipid syndrome;
  55. Disseminated Intravascular Coagulation
    • excessive and uncontrolled thrombosis leading to bleeding manifestations;
    • circulating plasma turns into serum;
    • "consumptive coagulopathy" - forming clots and consuming all of the products needed to form clots
  56. Hemophilia A
    • low FVIII; X-linked recessive only in males;
    • most common severe bleeding disorder
  57. Hemophilia B
    low FIX; X-linked recessive only in males
  58. Reticulocyte count will be low (<2%)
    Hypoproliferative Anemia
  59. Reticulocyte count will be high (>3%)
    Hyperproliferative Anemia
  60. seen primarily in southeast Asian, Mediterranean or African descent
    Alpha Thalassemia
  61. seen primarily in Mediterranean (Italian, Greek) and less often in Asians and Africans
    Beta Thalassemia
  62. fatigue, dyspnea on exertion, tachycardia, nail changes, dysphagia, pica ; microcytic/hypochromic as iron stores depleted
    Iron Deficiency Anemia
  63. mild to moderate anemia; initially normocytic and moves to mild microcytic hypochromic due to defect in moving iron into RBCs
    Anemia of Chronic Disease
  64. may have enlarged spleen or liver; mild to moderate anemia
    Sideroblastic Anemia
  65. glossitis, anorexia, diarrhea, peripheral neuropathy
    B12 Deficiency
  66. malnourished appearing, diarrhea, cheilosis, glossitis, NO neurological symptoms; alcohol is most common precipitator due to impaired hepatic function
    Folate Deficiency
  67. severe anemic symptoms
    Pure Red Cell Aplasia
  68. any age; pancytopenia; fatigue, weakness, excess bleeding/bruising, petechiae, purpura, pallor
    Aplastic Anemia
  69. anemia may not be apparent until RBC life span becomes shortened to approx 20 days
    Hemolytic Anemias
  70. anemia, splenomegaly, jaundice; pigment type gallstones may be present; maybe chronic leg ulcers
    Hereditary Spherocytosis
  71. episodic hemolytic anemia will cause Fe deficiency as well; venous thrombosis; deficient hematopoiesis causing pancytopenia; reddish-brown urine
    Paroxysmal Nocturnal Hemoglobinuria
  72. episodic hemolysis often triggered by oxidative stress (infections, toxins or drugs); no splenomegaly; no chronic hemolytic anemia
    Glucose 6 Phosphate Dehydrogenase Deficiency
  73. pallor, jaundice, splenomegaly, leg ulcers, pigment gallstones, priapism; vascular occlusion leading to infarction
    Sickle Cell Disorders
  74. anemia can be rapid onset and severe
    Autoimmune Hemolytic Anemia
  75. mottled or numb fingers/toes; anemia is rarely severe
    Cold Agglutinin Disease
  76. stomatitis (ulcers in mouth); fever sometimes; severe infections such as septicemia, pneumonia and cellulitis may occur
    Neutropenia
  77. dyspnea, headaches, visual disturbances, tinnitus, pruritus, thromboses; PE shows retinal-vein occlusion, ruddy cyanosis and splenomegaly
    Polycythemia Vera (PCV)
  78. rare in children, slight female predominance; approx 1/3 or pts asymptomatic at diagnosis; 2/3 of pts report vasomotor symptoms or complications from thrombosis or bleeding
    Thrombocytosis
  79. early stages asymptomatic; later stages: general malaise, weight loss, splenomegaly or splenic infarction (LUQ pain); hepatomegaly in 50% of pts
    Myelofibrosis
  80. asymptomatic; abnormal blood counts during routine screening; if symptomatic: fatigue, bleeding, recurrent infections, fever, splenomegaly, pallor
    Myelodysplastic Syndrome
  81. fatigue/anemia, recurrent infections, lytic lesions predisposing pts to bone pain, pathologic fractures and hypercalcemia, kidney failure, hyperviscosity of blood;
    Multiple Myeloma
  82. probable precursor to multiple myeloma
    Monoclonal Gammopathy of Unknown Significance (MGUS)
  83. fatigue, hyperviscosity syndrome (nausea, vertigo, visual, mucosal or GI bleeding); weight loss, headache, cold hypersensitivity, peripheral neuropathy, hepatomegaly, splenomegaly, engorged retinal veins
    Waldenström’s Macroglobulinemia
  84. male 2: female 1; 25% asymptomatic at diagnosis; fatgue, drenching night sweats, weight loss, frequent and/or persistent infections, lymphadenopathy (80%), skin infections/shingles less common
    Chronic Lymphocytic Leukemia
  85. young to middle age adults; phily chromosome present in over 95% of cases; bcr-abl is another way of diagnosing CML; fever with no infection, bone pain, LUQ pain due to enlarged spleen, fatigue, weakness, night sweats, bleeding and brushing, petechiae
    Chronic Myelogenous Leukemia
  86. pts acutely sick over days/weeks, not months; primarily children's disease; can affect T or B cells; malaise, fatigue, fever, bleeding gums, lympadenopathy, splenomegaly, petechiae, weight loss, meningitis, lethargy, anorexia, dyspnea
    Acute Lymphoblastic Leukemia
  87. similar to ALL; pancytopenia with circulating blasts, bone marrow with >20% blasts; peripheral smears showing WBC with Auer Rods; hepatomegaly, splenomegaly, elevated erythrocyte sedimentation rate
    Acute Myelogenous Leukemia
  88. median age of onset is mid 50s; male 5: female 1; fatique, abd discomfort from splenomegaly
    Hairy Cell Leukemia
  89. enlargement of lymphoid tissue, spleen, liver; painless cervical, supraclavicular, and mediastinal lymphadenopathy; constitutional/b symptoms (fever, drenching night sweats, weight loss); shortness of breath common with mediastinal mass
    Hodgkin's Lymphoma
  90. Three grades: indolent (slow growing); intermediate (aggressive, mix of small to large cells); high grade (very aggressive); lymphadenopathy persistent and painless, isolated or difuse; B symtpoms; occasional ab pain, vomiting, bleeding, edema
    Non-Hodgkin's Lymphoma
  91. usually in immunocompromised pts; organism in neutrophils and monocytes on Wright-Giemsa stained blood film; organism in macrophages on bone marrow smear
    Histoplasmosis
  92. nose bleeds, gum bleeds, bruising, menorrhagia, petechia
    Acute Thrombocytopenia
  93. same as acute (no systemic illness, spleen normal); common in childhood, starts with viral infection and have it intermittently throughout life; adult form is chronic
    Idiopathic (Immune) Thrombocytopenic Purpura
  94. heparin for 4-10 days (if plt count drops more by half or more than assume HIT); inflammation/necrosis at subcutaneous sites, limb asymmetry, cool/pulseless extremities; arterial or venous thrombosis
    Heparin Induced Thrombocytopenia
  95. mental status changes and symptoms correlating to end-organ damage (fever +/- mild renal insufficiency); associated with some meds; rare disease, but really sick; most prevalent in young women
    Thrombotic Thrombocytopenic Purpura
  96. renal failure; no mental status changes; most common in infants, children and pregnant women
    Hemolytic Uremic Syndrome
  97. hepatic coagulopathy
    Liver Disease (prolonged PT)
  98. bruises easily, increased bleeding after minor procedures (dental), menorrhagia*
    von Willebrand Disease
  99. can act in concert with smoking, birth control pills/hormone replacement
    Prothrombin 20210A Mutation
  100. anti-phospholipid (recurrent thromboses, 2nd trimester miscarriages, thrombocytopenia, valvular heart disease, migraines)
    anticoagulation disorders
  101. excessive thrombosis + bleeding (bc using up clotting products), organ dysfunction, shock, death; can get subacute DIC (Trousseau's syndrome) - see recurrent superficial cloting and DVTs
    Disseminated Intravascular Coagulation
  102. spontaneous hemarthroses
    Hemophilia A
  103. MCV < 80
    Microcytic Anemia
  104. MCV 80-100
    Normocytic Anemia
  105. MCV > 100
    Macrocytic Anemia
  106. A Thal trait: HgB electrophoresis shows no increase in HgB A2 or F, no HgB H; usually diagnosed via exclusion; Hgb H: peripheral smear abnormal, high retic count b/c hemolysis is occuring and body is trying to keep up with rate of destruction
    Alpha Thalassemia
  107. Thal major: severe anemia, marrow expansion causes bony deformities, growth retardations, hepato-splenomegaly (HSM), iron overload, predom Hgb F;
    Beta Thalassemia
  108. Thal intermedia: can have bone abnormalities and HSM, iron overload, 10% HgbA2 and the rest is HgbF; Thal minor: Hgb can be 3g/dL below normal, smear can show basophilic stippling
    Beta Thalassemia
  109. look at stool to rule out occult blood loss; classically microcytic, although Hgb will drop before MCV; abnormal iron indices, low ferritin, absent marrow iron, abnormal peripheral blood smear(anisocytosis),
    Iron Deficiency Anemia
  110. plts may be increased; (serum iron < 30, TIBC > 400, % sat < 10, ferritin < 15, marrow iron 0); RDW high; serum iron and ferritin low b/c used up stores
    Iron Deficiency Anemia
  111. microcytic or normocytic morphology; retic count usually normal; serum iron and TIBC may be low, ferritin is normal or elevated; marrow iron stores normal;
    Anemia of Chronic Disease
  112. LOW EPO LEVEL (bone marrow is sluggish because the pt has an underlying disease); MCV not as low as iron deficiency
    Anemia of Chronic Disease
  113. MCV usually low normal or high; serum iron high; diagnosed by bone marrow showing ringed sideroblasts
    Sideroblastic Anemia
  114. RBCs are macrocytic with MCV 110-140; anemia may be severe w/coexisting thrombocytopenia and leukopenia; peripehral smear may show hypersegmented neutrophils;
    B12 Deficiency
  115. decreased retic count; B12 levels <100pg/ml in symptomatic pts; elevated methylmalonic acid (indicator of deficiency at tissue level); Schilling's test
    B12 Deficiency
  116. macrocytic; similar to B12 deficiency (MCV high); RBC folate levels less than 150ng/mL; RBC folate is better than serum folate
    Folate Deficiency
  117. reticulocytes low or absent, morphology normal, WBC and plts not affected, bone marrow is normocellular
    Pure Red Cell Aplasia
  118. decreased retic count; morphology and MCV usually normal, but hematopoietic progenitor and precursor cells are less than 1% normal levels; bone marrow is hypocellular but no abnormal cells are seen
    Aplastic Anemia
  119. haptoglobin levels low; LDH often elevated; hemoglobinuria; elevated indirect bilirubin; elevated retic count; stable or falling Hgb
    Hemolytic Anemias
  120. loss of central pallor in RBCs; anemia usually microcytic and mild to moderate or absent (bone marrow is usually able to compensate); reticulocytosis is present; indirect bili may be elevated; Coombs test negative
    Hereditary Spherocytosis
  121. flow cytometry assays demonstrating absence of CD59 Ab; anemia is variable, usually normocytic unless Fe deficiency present; episodic hemolysis is intravascular so will find hemoglobinuria
    Paroxysmal Nocturnal Hemoglobinuria
  122. peripheral smear shows bite cells and Heinz bodies; reticulocytosis and increased bili during acute hemolytic episode; enzyme assays for G6PD will be low during an acute crisis
    Glucose 6 Phosphate Dehydrogenase Deficiency
  123. chronic hemolytic anemia, very low Hgb around 7-10g/dL; sicked cells in peripheral smear (5-50% of total RBCs); reticulocytosis; nucleated RBCs; leukocytosis and thrombocytosis; indirect bili is high; hemoglobin electrophoresis
    Sickle Cell Disorders
  124. positive Coombs test; severe anemia (Hgb 6-10g/dL); elevated retic count; peripheral smear shows spherocytes and nucleated RBCs; elevated indirect bili; 10% may have concurrent immune thrombocytopenia
    Autoimmune Hemolytic Anemia
  125. mild reticulocytosis; see spherocytes in peripheral smear; coombs test positive for complement; positive fold agglutinin test
    Cold Agglutinin Disease
  126. neutrophil count lower than 1500/mcL; calculate absolute neutrophil count (ANC) (the lower the ANC the greater the risk for serious, life-threatening infections)
    Neutropenia
  127. increased number of RBCs/Hgb and perhaps increase in other cell types (but not as great as increase in RBCs); splenomegaly, systolic hypertension, engorged retinal veins;
    Polycythemia Vera (PCV)
  128. positive JAK-2 serum test in 74-97% of pts (use to confirm dx); peripheral blood often appears microcytic with or without iron deficiency
    Polycythemia Vera (PCV)
  129. Physical exam often unremarkable but sometimes: splenomegaly, hepatomegaly, leukocytosis, erythrocytosis, mild anemia; occasional immature precursor cells and/or large plts in peripheral smear; increased number of megakaryocytes in bmbx
    Thrombocytosis
  130. plt count very high (over 600,000);
    Thrombocytosis
  131. fibrosis in bone marrow (difficult to aspirate), giant plts, teardrop poikilocytosis in advanced cases, nucleated RBCs (hematopoietic precursors) may be found in peripheral blood;
    Myelofibrosis
  132. hypercellular bone marrow with reticulin or collagen fibrosis; WBC counts usually increased but highly variable; plt counts initially may be high, normal or decreased
    Myelofibrosis
  133. SPEP IgG moderately elevated
    Monoclonal Gammopathy of Unknown Significance (MGUS)
  134. IgM spike (monoclonal antibody spike); anemia; plasmacytic lymphocytes on bmbx; differentiated from MGUS by the presence of bone marrow infiltration; serum viscosity; bone radiographs normal; low RBCs, low plts, high SPEP
    Waldenström’s Macroglobulinemia
  135. lymphocytosis (WBC > 20,000); anemia, thrombocytopenia, peripheral blood with mature small lymphocytes and smudge cells; hepatomegaly, splenomegaly, lymphadenopathy; coexpression of CD19 and CD5; hypogammaglobulinemia
    Chronic Lymphocytic Leukemia
  136. transition from low grade lymphoma/CLL to a high grade/aggressive lymphoma
    Richter’s transformation:
  137. B-symptoms:
    fever, wt loss, drenching night sweats
  138. most common presentation of non-Hodgkin’s lymphoma (could also be Hodgkin Disease or CLL)
    Painless, persistent lymphadenopathy
  139. Reed-Sternberg cells
    Hodgkin disease
  140. Ringed sideroblasts or 5q- syndrome
    Myelodysplastic Syndrome (MDS)
  141. Teardrop cells
    Myelofibrosis; Thalassemia major; Severe iron deficiency
  142. Elevated HGB/HCT
    PCV (polycythemia vera)
  143. Test to confirm a suspected diagnosis if CBC abnormal
    bone marrow biopsy
  144. Important chemo side effects:
    Adriamycin (cardiac toxicity)Vincristine (neuropathy)Bleomycin (pulmonary toxicity)
  145. Monoclonal IgM paraprotein
    Waldenström’s
  146. Rouleaux
    multiple myeloma
  147. Lytic lesions
    multiple myeloma
  148. Urinary Bence-Jones proteins or Serum M-spike proteins
    multiple myeloma
  149. Philadelphia Chromosome (bcr/abl gene rearrangement)
    CML (chronic myeloid leukemia)
  150. Imatinib (Gleevec & ST1571), oral chemo
    CML (chronic myeloid leukemia)
  151. Blasts + Pancytopenia (decreased WBC, RBC, and plts)
    acute leukemia (likely AML if adult, ALL if child)
  152. Auer Rods in WBCs =
    AML (Acute Myeloid Leukemia)
  153. Most common leukemia in children
    ALL (“all children”)
  154. Most common occurring leukemia
    CLL (Chronic Lymphocytic Leukemia)
  155. Smudge cells
    CLL (Chronic Lymphocytic Leukemia)
  156. Collective term for all physiologic mechanisms the body uses to protect itself from blood loss. Constant balance of maintaining blood in the fluid state & stopping bleeding from injured sites
    Hemostasis
  157. failure to maintain hemostasis
    Hemorrhage
  158. failure to maintain fluidity
    Thrombosis
  159. 4 Major Systems Involved in hemostasis
    Vascular System; Platelets; Coagulation System; Fibrinolytic System
  160. released, enhancing coagulation cascade & secondary (stable) hemostatic plug formation.
    PF3 (Platelet factor 3)
  161. potent stimulator of platelet aggregation & vasoconstriction
    thromboxane A2
  162. Intrinsic pathway: Factors
    12, 11, 9 & 8 (evaluated by PTT)
  163. Extrinsic pathway: Factors
    7 (evaluated by PT)
  164. Common pathway: Factors
    1, 2, 5 & 10 (PT & PTT)
  165. mild: no treatment; transfusion support; splenectomy; transplant; iron chelation therapy
    Thalassemia
  166. Hgb H: splenectomy may be helpful, folic acid to help with stability of RBC membrane
    Alpha Thalassemia
  167. Thal major: transfusions, splenectomy, iron chelation, allogeneic bone marrrow transplant for cure
    Beta Thalassemia
  168. evaluate and treat blood loss; oral iron supplements; encourage stool softeners; parenteral iron
    Iron Deficiency Anemia
  169. EPO injections + iron helpful for certain patients, but usually inadequate to maintain Hgb; transfusions are effective but multiple transfusions can generally cause problems down the road
    Anemia of Chronic Disease
  170. Deferoxamine (Desferal used at home, administered via IV or SQ for 12-16hrs/day; ascorbic acid (vit C) increases iron excretion used with deferoxamine (initiated 1-2 mos after chelator); deferasirox (Exjade) is an oral iron chelator but $$$
    thalassemia
  171. Anemia: morphology rel to MCV
    <80 = microcytic; 80-100 = normo; >100 = macro
  172. Diff Dx for micro anemia
    TICS: thal, iron def, chronic inflammation, sideroblastic
  173. Anemia: RBC production problem (vs destruction prob) distinguished by:
    • Retic count;
    • hypoproliferative = retic low (<2);
    • hyper = retic high (>3)
  174. Formula: corrected retic
    % retic x HCT/45% = absolute % retics
  175. Hypoproliferative anemia due to:
    • Marrow damage,
    • Fe def,
    • dec'd stimulation
  176. Hyperproliferative anemia due to:
    BM failure to compensate for blood loss or RBC destruction
  177. Degrees of thalassemia dz
    • Hydrops fetalis = all 4 genes deleted (fatal);
    • Hgb-H dz = 3 deleted (MCV=60-70);
    • A-thal trait = 2 genes (MCV=70-80);
    • silent carrier = 1 gene deleted
  178. Thal: Hgb-H dz labs
    • Hgb elect = 10-40% Hgb H;
    • retic is high
  179. Beta thal types
    • Major (Cooley's): both beta globin genes mutated, severe anemia, txn dept;
    • intermedia: txn in stress;
    • minor rarely need txn
  180. Tx for Hgb H dz
    Splenectomy? & folic acid
  181. Tx for beta thal major
    • Txn, splenectomy, Fe chelation;
    • allogeneic BM trans to cure
  182. Most common cause of anemia worldwide
    Fe def anemia
  183. Causes of Fe def anemia
    • Dec’d intake/ Poor diet;
    • dec’d absorption;
    • Inc’d loss (GI bleed, menorrhagia, neoplasm);
    • Inc’d reqs (PG, lactation)
  184. Tx for Fe def anemia
    Oral Fe supplement (ferrous sulfate)
  185. Anemia of chronic dz
    • Mostly normal labs (poss microcytic);
    • low erythropoietin;
    • tx underlying dz & coexisting defs; epo?
  186. Enzyme disorder: adequate Fe but can't get it into Hgb; Fe accumulates in ?
    • Sideroblastic anemia;
    • in RBC mitochondria
  187. Sideroblastic findings
    • High serum Fe, large spleen/liver;
    • mod anemia, low MCV;
    • BM sideroblasts
  188. Tx: Sideroblastic anemia
    • Dept on cause (ETOH, Pb, leukemia, TB Rx);
    • txn/chelation
  189. Macrocytic anemias
    Folic acid def; B12 def
  190. Causes of B12 def
    • Diet (vegans);
    • malabsorption: inadequate IF prodn (70%), terminal ileum dz (sprue, Crohns), tapeworm, drugs
  191. S/S = Glossitis, periph neuropathy (Stocking-glove paresthesias); MCV 110-140; low retic, hyperseg PMNs, high methylmalonic a.
    B12 def
  192. Tx B12 def
    • Replacement tx: IV B12;
    • frontload dose
  193. B12 def vs folic acid def
    • B12: meat/dairy;
    • to 5000 mcg in liver (3 yrs);
    • Folic acid: fruit/veg; 5-20 mg stored (few mos)
  194. Causes folic acid def
    • Inadeq intake (ETOH, teens, elder);
    • inc’d req: PG, malignancy, anemias, hemodialysis;
    • Malabsorption [sprue, drugs (phenytoin, barbiturates)];
    • Impaired metabolism (ETOH, methotrexate)
  195. Folic acid def findings
    • Malnourished;
    • glossitis,
    • cheilitis;
    • NO neuro S/S;
    • RBC folate <150 ng/mL
  196. PRCA is contracted:
    Acquired or idiopathic
  197. Autoimmune dx mediated by T lymphocytes or (rarely) IgG antibody against erythroid precursors
    PRCA
  198. Poss causes include thymoma, solid tumors, SLE, RA, HIV/hep, phenytoin
    PRCA
  199. PRCA findings
    • Normal lab except low retic;
    • severe anemia S/S
  200. PRCA Tx
    • Stop all meds;
    • poss thymoma resection;
    • IV Ig
  201. Due to BM failure (injury or suppression); pancytopenia
    Aplastic anemia
  202. Causes of aplastic anemia
    • Idiopathic (50-65% ; likely autoimmune);
    • Phenytoin, sulfonamides;
    • chemotherapy, radiotherapy;
    • benzene, solvents, insecticides;
    • hep, HIV, EBV; PG, SLE, GVHD;
    • Hereditary (rare): Fanconi’s anemia
  203. Aplastic anemia findings
    • NO hepatosplenomegaly;
    • pancytopenia (purpura, pallor, petechiae);
    • low retic;
    • hypocellular BM
  204. Aplastic anemia: diff dx from: 1-MDS; 2-hairy cell; 3-normo
    • 1=abn cells;
    • 2=splenomegaly & abn lymph cells;
    • 3=SLE, hypersplenism, dissem'd infxn
  205. Aplastic anemia tx
    • Family HLA typing;
    • supportive tx (txn/infxn);
    • immunosuppn;
    • GF;
    • BM txplant
  206. Aplastic anemia prognosis
    • Untx'd = fatal;
    • HLA matched txplant: 60-90% cure;
    • ATG tx: partial remission 60-80% (1/3 relapse & 20-50% MDS)
  207. Hemo anemias: intrinsic vs extrinsic
    • Intrinsic = prob w/membrane, z defects, hgb (usu hereditary);
    • Extrinstic = autoimmune, drugs or mechanical trauma (often acquired)
  208. Reticulocytosis may reflect:
    Hemolytic anemias
  209. Hemo anemia lab findings
    Low haptoglobin; high LDH, indirect bili (not >4-5mg/dL unless underlying liver dz), retic; hemoglobinuria; stable or falling hgb
  210. Distinguishing Etiology of Hemolysis
    • Clinical & FH;
    • Direct Coombs;
    • Peripheral blood smear;
    • morpho (Heinz bodies, sickle cells, parasites);
    • Hgb electrophoresis
  211. Due to spectrin pro in cytoskeleton
    Hereditary spherocytosis
  212. Triad: anemia, splenomegaly and jaundice
    Hereditary spherocytosis
  213. Pigment type gallstones; chronic leg ulcers
    Hereditary spherocytosis
  214. Hereditary spherocytosis findings
    • Mod micro anemia;
    • reticulocytosis;
    • high indirect bili;
    • Neg Coombs
  215. Hereditary spherocytosis tx
    • Folic acid 1mg po qd;
    • splenectomy for severe
  216. Acquired stem cell disorder making RBC membrane prone to lysis by complement
    PNH
  217. PNH findings
    • Episodic hemolytic anemia;
    • Venous thrombosis;
    • Deficient hematopoiesis -> pancytopenia;
    • reddish-brown urine;
    • Hemoglobinuria, esp. first morning urine
  218. Episodic hemolytic anemia; Venous thrombosis; Deficient hematopoiesis -> pancytopenia ... indicate:
    PNH
  219. PNH labs
    • Flow cyto = absence of CD59 Ab;
    • normocytic;
    • hemoglobinuria
  220. In PNH, increased risk of:
    leukemia/myelofibrosis
  221. PNH tx
    • Tx Fe def;
    • prednisone for hemolysis;
    • BM txp if severe
  222. Episodic hemolytic anemia related to oxidative stress (hereditary)
    G6PD def
  223. G6PD def prevalence
    • >200M worldwide (usu AA men);
    • X linked recessive;
    • females rare
  224. Stress triggers of G6PD def
    • Infxns;
    • toxins/drugs (sulfa/antimalarial);
    • fava beans
  225. G6PD def findings
    • No splenomegaly or chronic hemolytic anemia;
    • bite cells/Heinz bodies;
    • Reticulocytosis and inc bili in crisis;
  226. G6PD def tx
    • Splenectomy for severe; (txn rarely);
    • avoid stressors (ASA,sulfa, antimal, fava)
  227. Autosomal recessive disorder in which abnormal Hgb leads to chronic hemolysis
    Sickle cell disorders
  228. In sickle cell dz, rate of sickling depends on:
    Conc of Hgb S, dehydration, presence of other Hgb (F), hypoxemia & acidosis
  229. Sickle cell prevalence
    • 8% of AA: Hgb S gene;
    • 1/400 AA have SSA;
    • onset in 1st yr (Hgb F falls);
    • 40-50 yr life exp
  230. S/S = Pallor, jaundice, splenomegaly, leg ulcers, pigment gallstones, priapism
    Sickle cell
  231. 2 RBC conditions w/pigment gallstones
    Hereditary spherocytosis, sickle cell
  232. Sickle cell: vascular occlusion leading to infarction
    • Pulmonary (acute chest syndrome);
    • Retinal (blindness?);
    • Renal (to renal failure );
    • Stroke, even in kids;
    • Splenic infarcts;
    • aseptic necrosis of femoral head
  233. Sickle cell labs
    • v low hgb (7-10);
    • sickled cells = 5-50% of RBCs;
    • reticulocytosis; NRBCs;
    • Leukocytosis, thrombocytosis, high indirect bili
  234. Sickle cell tx
    • Folic acid 1 mg po qd;
    • hydrate, low alt, vax (flu/pneumo/mening)
  235. SSA longterm issues
    • Splenectomy: infxn probs;
    • narco abuse;
    • Hydroxurea to inc hgb F
  236. Acquired disorder which IgG autoantibody formed that binds to RBC membrane
    AIHA (autoimmune hemo anemia)
  237. AIHA: ___% are idiopathic; seen in ___
    50% ; lupus, NHL, CLL
  238. AIHA findings
    • Pos coombs test;
    • severe anemia (6-10);
    • high retic & ind bili;
    • spherocytes & NRBC;
    • Evans syndrome (immune thrombocytopenia)
  239. AIHA tx
    • Prednisone 1 mg/kg/d;
    • splenectomy if fail;
    • Rituximab, Danazol, IVIG if fail;
    • avoid txn
  240. Acquired hemolytic anemia due to IgM autoantibody
    Cold agglutinin dz
  241. Cold agglutinin dz causes
    Idiopathic or neoplasm or post-infxn (Monoclonal gammopathy, mono, mycoplasma)
  242. Cold agglutinin dz findings
    • Mottled/numb fingers/toes;
    • anemia is rarely severe;
    • mild reticulocytosis;
    • spherocytes;
    • Pos Coombs for complement;
    • Pos cold agglutinin test;
    • Chilled blood will look clumped on slide
  243. Cold agglutinin dz tx
    • Symptomatic, avoid cold;
    • poss Rituximab or immunosupp or plasmapheresis;
    • no splenectomy/steroid
  244. Defn neutropenia
    PMN <1500 (AA may have 1200 normal)
  245. Absolute Neutrophil count formula
    WBC x (% segs + % bands) = ANC
  246. Neutropenia findings
    • Stomatitis;
    • fever;
    • poss septicemia, pneumonia and cellulitis
  247. Neutropenia tx
    • DC potl causative agents;
    • tx infxns;
    • poss GF (G-CSF) for severe
  248. Anemia: no hepatosplenomegaly
    aplastic anemia
  249. Anemia: No neuro S/S
    Folic acid def
  250. Aplastic anemia S/S
    No hepatosplenomegaly
  251. Reticulocytosis
    hemolytic anemias
  252. Neg Coombs
    hereditary spherocytosis
  253. Pos Coombs
    AIHA, cold agglutinin
  254. Heinz bodies
    Hemolytic anemias (G6PD)
  255. Low retic
    • hypoproliferative anemia;
    • B12;
    • PRCA;
    • aplastic anemia
  256. Evans syndrome
    (immune thrombocytopenia) AIHA
  257. Anagrelide may be used for:
    P vera; thrombocytosis
  258. Cytoreductive tx
    • (eg, thrombocytosis);
    • Anagrelide, hydroxyurea
  259. Engorged retinal veins
    P vera; Waldenström
  260. Causes of vessel wall dz
    • Scurvy (Vit C def);
    • Amyloidosis;
    • Hereditary-Hemorrhagic Telangiectasia (Osler-Weber-Rendu Dz);
    • Neurofibromatosis;
    • Collagen Dz (Marfan; Ehler-Danlos)
  261. Causes of thrombocytopenia (decrease in plt numbers)
    • Decreased prodn (BM prob);
    • inc destn (meds, immune mediated, DIC);
    • Sequestration (Splenomegaly)
  262. Causes of decreased plt function
    • Membrane Defects (Glanzmanns Thrombasthenia; Bernard-Soulier);
    • Pathway Defects (Arachidonic Pathway Defect; Aspirin/Clopidogrel Use);
    • Others (MDS)
  263. Examples of plt dz
    • Acute Thrombocytopenia;
    • HIT;
    • ITP;
    • TTP;
    • HUS
  264. ITP dx is...
    one of exclusion (i.e., notable for what is not present)
  265. ITP findings
    • Neg FH/SH/PMH;
    • labs = acute thrombocytopenia;
    • all else normal
  266. ITP tx
    • Immunosuppression (steroids, Rituximab, cyclophosphamide );
    • Immune Modulation (IVIg, Splenectomy);
    • Stim of Plt Prodn (Eltrombopag, Romiplostim)
  267. 2 types of HIT
    • 1-transient decrease;
    • 2-immune mediated (sig)
  268. HIT mechanism
    • Heparin combine w/PF4;
    • this plus Abs -> plt activation -> limb or life threatening thrombosis
  269. HIT PMH
    • On heparin 4-10 days;
    • Inflammation/necrosis?;
    • limb asymmetry? ;
    • Cool/pulseless extremities?
  270. HIT labs
    • Plt drop 4-10 d post heparin;
    • 50% of plt baseline;
    • coags normal;
    • confirm w/Serotonin Release Assay
  271. HIT tx
    • Stop all heparin products (inc hep lines & flushes & LMWHs);
    • prompt tx w/alt. anti-coagulation: Direct Thrombin Inhibitor (Argatroban, Lepirudin, Bivalirudin);
    • Fondaparinux (synthetic anti-coagulant);
    • warfarin after plt normal;
    • w/alt anticoag;
    • 6-12 wks
  272. Dz = increase in ultra-high molecular weight multimers of von Willebrand Factor
    TTP
  273. TTP causes:
    • Intra-vascular platelet aggregation;
    • systemic manifestations (CNS, Renal, Cardiac, Hematologic)
  274. H&P for TTP
    • mental status changes;
    • S/S rel to end-organ damage;
    • other=sim to acute & idio thrombocytopenia
  275. TTP labs
    • Plt usu <20K;
    • coags/labs normal;
    • micro-angiopathic hemolytic anemia (MAHA)
  276. micro-angiopathic hemolytic anemia (MAHA)
    • TTP;
    • anemia, hemolysis, schistocytes
  277. TTP tx
    • Plasma exchange (lg bore catheter):
    • blood removed, plasma sep’ & discarded, RBCs returned to pt;
    • fresh plasma given back to pt;
    • Immune Suppression(?)
  278. HUS: mental status changes?
    No
  279. HUS findings
    • Thrombocytopenia;
    • labs = TTP
  280. HUS tx
    • Supportive;
    • plasma exchange?
  281. Extrinsic & intrinsic coag pathways converge to form:
    factor Xa
  282. PT measures:
    extrinsic (FVII) & common pathways (Factor 1,2,5,10)(exogenous TF is added to initiate clotting);
  283. PT is sensitive to (1) & normal range is (2)
    • (1) FVII ;
    • (2) 9.5-13.1
  284. aPTT measures:
    intrinsic (factors 12,11,9,8) & common pathways (1,2,5,10)
  285. aPTT normal range
    24.1-32.3 sec (uses neg charged particles to initiate)
  286. Cause of prolonged PT:
    • Def. in FVII and Common Pathway Factors (FX, FV, FII, Fibrinogen);
    • Liver Dz;
    • Warfarin tx;
    • Vit K Def;
    • Inhibitor to FVIIa (rare)
  287. Very rare causes of prolonged PT
    • FVII def;
    • FVIIa inhib
  288. Common pathway defs will prolong:
    PT & aPTT
  289. All clotting factors (x FVIII) made in:
    Liver
  290. fx of liver dz on coag
    • 1st: dec in FVII (short half-life) prolongs PT;
    • then dec in other factors -> prolonged aPTT;
    • dz causes splenomegaly -> thrombocytopenia
  291. MOA: warfarin tx on high PT
    Warfarin antagonizes the action of Vitamin K
  292. Vitamin K is a necessary co-factor in the g-glutamyl carboxylation of:
    Factors II, VII, IX, X; also Protein C and Protein S
  293. MOA: vit K def on high PT
    Sim. to warfarin
  294. Situations in which vit K def is seen:
    • HDN (tx w vit K);
    • malabsorption (vit K, A,D,E fat soluble);
    • malnutrition; antibx use
  295. Causes of prolonged aPTT:
    • Def of intrinsic (FXI, FVIII, FIX ) & common pathway factors;
    • Heparin & DTI tx;
    • Inhibitors to coag factors
  296. Deficiencies in HMWK, PK, and FXII are assoc w/which bleeding problems?
    Deficiencies in HMWK, PK, and FXII are NOT associated with any bleeding
  297. Clotting factor def assoc w/Jewish ancestry
    FXI def
  298. FVIII def AKA = ? Prevalence = ?
    • Hemophilia A;
    • 1:5,000 live births;
    • primarily in males
  299. FIX Deficiency AKA = ? Prevalence = ?
    • Hemophilia B;
    • 1:30,000 live births;
    • primarily in males
  300. FVIII, FIX Deficiency: percentages assoc w/bleeding/severity
    • <1% assoc w/ spontaneous bleeding;
    • 1-5% moderate, can still have spont bleed;
    • 5-40% can bleed w/procedures/trauma
  301. Hx includes hemarthrosis, bleeding into joints, and/or x-linked FH of probs
    FIXdef; FVIII def
  302. FVIII/FIX def findings
    Labs: aPTT prolonged; FVIII/FIX levels decreased
  303. FVIII/FIX def tx
    Specific Factor Replacements: FVIII or FIX Concentrate; plasma derived or recombinant
  304. Prolonged aPTT; doesn't correct w/mixing study
    FVIII inhibitor
  305. FVIII Inhibitor
    • Seen in PG & the elderly;
    • Presents with provoked – spontaneous bleeding;
    • Rare ( 1:1,000,000);
    • Auto-Ab vs FVIII, prevents fn or increasing clearance
  306. FVIII Inhibitor
    • Seen in PG & the elderly;
    • Presents with provoked – spontaneous bleeding;
    • Rare ( 1:1,000,000);
    • Auto-Ab vs FVIII, prevents fn or increasing clearance
  307. Treatment to Eradicate FVIII Inhibitor:
    • Steroids +/- Cyclophosphamide.
    • Tx if Bleeding: If inhibitor is of low titer, can give FVIII;
    • if titer is high, give FVIIa
  308. von W Dz usu presents w/PT/aPTT = ?
    Normal
  309. Most common bleeding disorder, at a prevalence of ?
    vW Dz (1%)
  310. limited to mucocutaneous bleeding
    vW Dz
  311. Fns of vW factor
    • Bridges plts;
    • w/endothelium;
    • Bridges plts together;
    • Carrier for FVIII, extending its half-life
  312. vW Dz lab data
    • Low levels of vW antigen (VWF:Ag);
    • Low levels of vW Activity (VWF:RCo);
    • Multimers are decreased
  313. vW Dz tx (for mild Forms):
    • ddAVP (synthetic vasopressin);
    • intranasal or IV; -> inc. release of pre-formed vWF (Only works for a day or two);
    • e-aminocaproic acid (Works by inhibiting fibrinolysis)
  314. vW Dz tx (for Severe Bleeding/Surgery):
    • Replace with VWF:FVIII containing concentrates;
    • Humate-P
  315. Coagulation dz: causes elevated levels of prothrombin; leads to a modest increase in hypercoagulability, (2-fold)
    Prothrombin 20210A Mutation
  316. Second most common inherited cause of hyper-coaguability
    Prothrombin 20210A Mutation
  317. Prothrombin 20210A Mutation: concomitant risk factors
    Smoking, BPCs/Hormone replacement
  318. Fibrinolysis/Anti-coagulant Disorders usu present as:
    thrombosis
  319. Antithrombin activity is markedly stimulated by:
    Heparin
  320. Antithrombin def is manifested by:
    Recurrent venous thromboses
  321. Protein C or Protein S Def: Predisposition to:
    recurrent venous thromboses
  322. Uncommon causes of venous thromboses
    • Antithrombin def;
    • Protein C or Protein S Def
  323. Activated Protein C Resistance AKA:
    Factor V Leiden
  324. Dz: mutation makes it resistant to inactivation by Activated Protein C
    Factor V Leiden
  325. Most common hereditary hyper-coagulable state; mainly ltd to ?
    • Factor V Leiden;
    • pts of Euro extraction
  326. Factor V Leiden: Heterozygous state increases risk of thrombosis up to:
    8-fold
  327. Factor V Leiden: Homozygous state increases risk:
    100-fold
  328. Auto-antibody that interferes with the natural anti-coagulant system
    Anti-Phospholipid Syndrome
  329. Anti-Phospholipid Syndrome presents with:
    • Recurrent arterial or venous thromboses;
    • 2nd trimester miscarriages;
    • Thrombocytopenia;
    • Valvular heart dz
  330. Recurrent arterial or venous thromboses; 2nd trimester miscarriages; Thrombocytopenia; Valvular heart dz; suggests ?
    Anti-Phospholipid Syndrome
  331. Anti-Phospholipid Syndrome: Lab Data:
    • Presents w/prolonged aPTT (b/c in vitro fx of the Ab on aPTT);
    • mixing study fails to normalize;
    • Confirmatory tests required to make the diagnosis
  332. Anti-Phospholipid Syndrome: tx
    Once thrombotic event occurs, tx w/warfarin indefinitely
  333. Condition in which circulating plasma (which contains the clotting factors), turns into serum
    DIC
  334. DIC: presentation
    • Excessive Thrombosis;
    • Organ Dysfunction (Renal; Hepatic; CNS);
    • Bleeding, Shock, Death
  335. Acral ischemia
    DIC
  336. Hx pos for Heparin, Tacrolimus, Ticlopidine may point to:
    DIC
  337. Signs of hemorrhage in DIC
    • Mucous membranes;
    • IV Sites, Catheters, ETT;
    • Venipuncture sites
  338. CBC for pt w/DIC
    WBCs up or down; probably toxic granulations; anemia, thrombocytopenia; schistocytes
  339. DIC labs:
    • Coags: PT prolonged (first one to go “out” -- due to very short half-life of FVII);
    • aPTT prolonged;
    • factor levels decreased (II, V, VII, VIII, IX, X);
    • Fibrinogen (AKA Factor I) decreased:
    • AT decreased;
    • D-Dimer elevated
  340. DIC tx
    Tx underlying dz
  341. DIC tx (if bleeding, or surg needed)
    • Plts;
    • blood;
    • Fibrinogen, if <100 (Give cryo; theoretical concern over worsening thrombosis if given alone, since cryo lacks AT, Protein C, and Protein S)
  342. Defn Hemorrhage:
    failure to maintain hemostasis
  343. Defn Thrombosis:
    failure to maintain fluidity
  344. Steps in forming the primary hemostatic plug:
    • Plt Adhesion;
    • plt Aggregation;
    • plt surface activation & recruitment of other plts;
    • plt Release
  345. Platelet Release involves:
    • Release of PF3 (Platelet factor 3);
    • intracellular Ca (enhances further activation);
    • thromboxane A2
  346. thromboxane A2 is:
    potent stimulator of plt aggregation & vasoconstriction
  347. visible end product of coagulation =
    Fibrin
  348. fibrin is digested by
    plasmin
  349. Normal BT is
    < 8 min
  350. Single best indication of functional platelet deficiency
    Bleeding time
  351. BT prolonged when:
    • Plts <100,000;
    • abnormal plt fn;
    • drugs like Plavix
  352. Adult ITP
    • usu chronic (aot kids, no viral infxn);
    • peak = 20-50 y.o.; F:M = 2:1
  353. Most common drug causing ITP
    Heparin
  354. Dz’s causing ITP
    SLE, CLL, virus (HIV/Hep C)
  355. TTP findings
    • High LDH;
    • neuro & renal S/S;
    • MAHA;
    • schistocytes;
    • AutoAb to ADAMTS13
  356. Dx sim to TTP but w/o neuro S/S
    HUS
  357. AutoAb to ADAMTS13 seen in:
    TTP
  358. TTP tx:
    plasmapheresis
  359. HUS tx & prognosis
    • plasmapheresis FFP;
    • kids self-limiting;
    • 40% adults die;
    • 80% get renal insuff (CRI)
  360. Trousseau’s syndrome
    Subacute DIC; seen in cancer pts
  361. DIC labs
    • low plt & fibrinogen;
    • schistocytes;
    • pos D-dimer (more spec than pos FSP);
    • PTT prolonged more often than PT (both may be abn)
  362. DIC tx
    • tx underlying condition;
    • replace blood products PRN (platelets, FFP, cryo, PRBCs, AT3);
    • poss heparin or LMWH
  363. most common congenital disorder of hemostasis
    von Willebrand’s
  364. von Willebrand’s FH
    autosomal dominant
  365. von W defect is in:
    platelet adhesion only
  366. vWF is synthesized in:
    endothelial cells & megakaryocytes
  367. Fn of vWF:
    mediate platelet adhesion to damaged vessel site
  368. vWF is carrier protein for:
    • factor 8;
    • vWF pts have decreased factor 8 levels & prolonged PTT
  369. vWF labs
    • BT > 8 min;
    • low factor 8 level; high PTT;
    • plt aggn abn w/ ristocetin;
    • low vW Ag
  370. vW dz Rx
    • avoid aspirin products;
    • may use DDAVP (also use pre-operatively)
  371. Glanzmann’s
    • rare autosomal rec;
    • def in IIb/IIIa, plts can’t agg;
    • high BT
  372. Glanzmann’s tx
    plt transfusion
  373. Liver = site of prodn of all clotting factors except:
    factor 8
  374. Thrombopathies
    • Von Will;
    • Glanzmann’s thrombasthenia;
    • Bernard-Soulier;
    • Acquired
  375. Tx for liver dz/thrombocytopenia
    FFP
  376. Bernard-Soulier Syndrome
    • Rare autosomal rec;
    • low/abn plt vWF receptor;
    • giant plts, thrombocytopenia, prolonged BT
  377. Platelet aggregation abnormal with ristocetin, which corrects with the addition of normal platelets, in:
    Bernard-Soulier Syndrome
  378. Causes of acquired thrombopathy
    • Drugs (ASA, NSAIDS, cephalosporins);
    • ETOH (>BT, >*PT, PTT also prolonged in end-stage liver dz);
    • Renal dz (unknown mechanism);
    • Dysproteinemias or MM
  379. Defn Thrombopathy (Qualitative)
    Bleeding disorders characterized by prolonged BT despite normal platelet count
  380. Subacute DIC (Trousseau’s syndrome): will see:
    • Recurrent superficial & DVTs;
    • low plt & high D-dimer
  381. ___ pathway initiates coag process; then _____ pathway enhances _____ formation in clot
    • Extrinsic;
    • intrinsic;
    • thrombin
  382. First factors to be affected in hepatic dysfunction
    Vit K dependent factors (2, 7, 9 & 10) + factor 5
  383. natural anticoagulants
    Proteins C & S
  384. causes of vit K def
    • biliary tract dz;
    • drugs;
    • malnutrition
  385. Most common drug induced form of thrombocytopenia
    HIT
  386. HIT occurs when?
    4-10 days post hep infusion
  387. HIT involves:
    IgG antibody to Platelet Factor 4
  388. Confirm HIT with:
    Serotonin Release Assay
  389. Unlike PTT, PT tests for
    factor 7
  390. PT monitors:
    • Coumadin;
    • common pathway (1,2, 5, 10); F7
  391. PT/PTT ref ranges
    • PT = 11-13;
    • PTT = 26-36
  392. PTT monitors:
    • Heparin;
    • intrinsic (12, 11, 9, 8);
    • common pathway (1,2, 5, 10)
  393. Heparin tx PTT range
    50-70 s
  394. Fibrinogen unique to:
    • plasma;
    • ref range = 206-382
  395. Factor Xa to monitor:
    LMWH (eg, enox/Lovenox)
  396. Hereditary hypercoag states
    Factor V Leiden; Protein C & S def; AT III deficiency
  397. Hemophilia A
    • X linked rec;
    • males 1:10K;
    • hemarthroses;
    • low FVIIIc;
    • high PTT;
    • tx = FVIII, DDAVP
  398. Most common severe bleeding disorder
    Hemophilia A
  399. Hemophilia B
    • Xmas dz;
    • X linked rec;
    • males;
    • low FIX;
    • high PTT;
    • tx = FIX, NO DDAVP
  400. Lupus Anticoagulant
    • Circulating IgG or IgM;
    • high PTT (no correction with mixing studies);
    • risk of thrombosis or recurrent spontaneous abortion
  401. Most frequent cause of hereditary thrombophilia
    • Factor V Leiden (AKA APC);
    • hereditary resistance to action of activated protein C (4-8% of pop)
  402. Diff btw hep & LMWH
    • Hep binds to anti-thrombin III (inactivate coag factors);
    • LMWH binds to ATIII and directly inactivates Xa
  403. D-Dimer
    • Nonspecific;
    • only neg test is helpful; PE;
    • 90% pts w/thrombotic & thromboembolic dz;
    • Neg <0.349;
    • Low Pos = 0.350-0.500;
    • Positive = >0.500
  404. Protein C def pts
    • warfarin hyper-sensitivity rxn;
    • may have to decrease coumadin by half (lest get necrotizing condition)
  405. Type I HIT:
    • Non-Immune mediated;
    • plt = 100,000, no thrombosis;
    • D/C heparin, counts normalize
  406. Type II HIT:
    • Immune mediated;
    • plt = 50,000, hi risk of thrombosis;
    • D/C Heparin, Rx direct thrombin inhibitors, then Coumadin
  407. ACT
    • Activated clotting time;
    • monitors heparin in OR (check if safe to pull cath/A lines);
    • Normal blood clots in <100 seconds;
    • Heparin therapeutic = 300-600 s
  408. Distinguish liver dz coagulopathy from vit K def
    • Vitamin K def only: 5 is normal, 7 is low;
    • Liver dz only: both 5 & 7 are low
  409. Deficiency allows unopposed conversion of fibrinogen to fibrin
    ATIII def
  410. Virchow's Triad
    • Vascular damage;
    • Hypercoagulability; Vascular Stasis;
    • Predisposes patient to venous thrombosis
  411. Pathophysiologic Basis for Myeloproliferative Disorders
    • Acquired clonal abnormalities of the hematopoietic stem cell;
    • May see changes in all stem cell lines (erythroid, myeloid, & pt cells);
    • poss specific chromosomal changes
  412. True increase in RBC mass
    Primary P. vera
  413. Relative increase in RBC mass
    Secondary P. vera
  414. Conditions causing secondary P. vera
    Conditions such as hypoxia (COPD, heart disease, cigarette smoking), renal disease, MI’s, dehydration, high altitude, autotransfusion (blood doping
  415. P. vera: incidence/prevalence
    • 60 to 70 yrs (mean age at dx: 65 yrs; rare in pt <40);
    • M/F = 1.2 to 1
  416. P. vera S/S
    Probs rel to hyperviscosity & hypervolemia; pruritus (esp. after hot showers/baths), thromboses; dyspnea; HA; visual disturbance; tinnitus
  417. Hallmark of P. vera =
    Erythrocytosis
  418. P. vera H & H
    • M: >18.5 ;
    • F: >16.5 (often HCT is > 60%)
  419. Condition w/thrombosis; splenomegaly; Plethora; systolic hypertension; engorged retinal veins
    P. vera
  420. P. vera lab data
    Positive JAK-2 (janus kinase) serum test (pos in 74-97% of pts)
  421. P. vera mgmt
    • Tx phlebotomies (Hct <45 % M; Hct <43% F);
    • myelosuppression if nec;
    • hydroxyurea (watch WBC/PLT);
    • Anagrelide if thrombocytosis;
    • Aspirin 81mg to reduce thrombosis risk;
    • antihistamine for pruritus
  422. P. vera mgmt
    • Tx phlebotomies (Hct <45 % M; Hct <43% F);
    • myelosuppression if nec;
    • hydroxyurea (watch WBC/PLT);
    • Anagrelide if thrombocytosis;
    • Aspirin 81mg to reduce thrombosis risk;
    • antihistamine for pruritus
  423. P. vera prognosis
    • Median Survival Tx: 11-15 yr;
    • untx’d = 18 mos;
    • 5-20% evolve into myelofibrosis or acute leukemia over 20 yrs
  424. Causes of Reactive (Secondary) Thrombocytosis
    • Severe hemorrhage;
    • splenectomy;
    • neoplasms;
    • chronic inflame dz;
    • post acute infxn;
    • B12 def;
    • meds (vincristine, epi);
    • ETOH
  425. Causes of Reactive (Secondary) Thrombocytosis
    • Severe hemorrhage;
    • splenectomy;
    • neoplasms;
    • chronic inflame dz;
    • post acute infxn;
    • B12 def;
    • meds (vincristine, epi);
    • ETOH
  426. Thrombocytosis S/S
    • Median 50-60 yrs (but all ages);
    • rare in kids;
    • slight female predominance;
    • 1/3 of pts asymptomatic at dx;
    • 2/3 of pts vasomotor s/s (HA, dizziness, visual changes) or complications from thrombosis/bleeding
  427. Thrombocytosis findings
    • Splenomegaly (25+% );
    • hepatomegaly (20 %);
    • Leukocytosis, erythrocytosis, mild anemia;
    • occ immature precursor cells and/or large plts;
    • BM bx: inc no. of megakaryocytes, else normal
  428. Thrombocytosis Mgmt
    • ASA to prevent thrombosis;
    • Cytoreductive tx (hydroxyurea, anagrelide);
    • Plt pheresis if severe bleed;
    • BM bx for Philadelphia chr.
  429. Thrombocytosis Prognosis
    • 10 y survival = 64-80% ;
    • 1-5% evolve into AML, 10-15% evolve into myelofibrosis
  430. Disorder in which bone marrow is replaced with scar tissue, leading to anemia
    Myelofibrosis
  431. Myelofibrosis findings
    • Fibrosis on BM;
    • splenomegaly;
    • giant plts;
    • teardrop poikilocytosis
  432. Myelofibrosis peak incidence & survival
    • 50-70 yrs old;
    • median survival is 2-5 yr from onset;
    • occurs in 10 to 30% of pts w/P. vera
  433. Cause of Myelofibrosis
    Unknown
  434. JAK-2 mutations may be associated with:
    • Myelofibrosis, thrombocytosis;
    • P vera = often pos JAK-2 serum test
  435. Increased bone marrow production of megakaryocytes leads to increased peripheral platelet count
    Myelofibrosis
  436. Myelofibrosis S/S
    Early: asymptomatic; later: malaise; wt loss; splenomegaly/splenic infarction; hepatomegaly in 50% of pts
  437. Myelofibrosis findings
    • BM aspirate = dry tap;
    • anemia generally increases over time;
    • normochromic-normocytic & mild poik;
    • NRBCs
  438. Myelofibrosis mgmt
    • No tx to reverse/ctrl underlying pathology;
    • tx supportive;
    • mgmt of complications; (Procrit, Aranesp);
    • pRBC & plt txn;
    • Thalidomide & Revlimid? ;
    • allogeneic BM txplt for younger pt? ;
    • Median survival 5 yrs
  439. Acquired clonal disorders of the hematopoietic stem cell
    MDS
  440. ______ cytopenias affect one or more cell lines (RBC, WBC, and/or PLTs)
    MDS
  441. Dz occurs when blood cells do not develop into mature cells, but rather stay in an immature stage within the BM
    MDS
  442. Some chromosomal abnormalities (5q – loss of part of the long arm of chromosome 5); “pre-leukemia”
    MDS
  443. MDS: risk factors include exposure to:
    Benzene, radiation, chemotx agents (esp alkylating agents & anthracyclines)
  444. MDS pts
    • Average age ≥ 60 years;
    • pts often asymptomatic;
    • If S/S: fatigue, bleeding, recurrent infxn, fever, splenomegaly, pallor
  445. MDS Labs
    85% of pts anemic; 50% neutropenia; 30% thrombocytopenia
  446. MDS CBC
    • Normal or low RBC, WBC, PLTS;
    • Blasts in BM <20%;
    • Pelger-Huet cells (bi-lobed neutrophils)
  447. MDS: >20% blasts in BM indicates:
    Transition into acute leukemia
  448. Blasts in BM <20%; Pelger-Huet cells (bi-lobed neutrophils) seen in:
    MDS
  449. MDS BM shows:
    Hypercellular marrow with delayed/abnormal maturation ( 5q- chromosomal abnormality, ring sideroblasts)
  450. MDS Mgmt
    • Monitor closely (lest transformation);
    • Cytokine and transfusion support;
    • chemotherapy (Thalidomide, Lenolidimide for 5q- syndrome, Azacitadine);
    • Allogeneic BM txplt only curative therapy (most pts too old)
  451. MDS: Allogeneic BM txplt may cure ?? % of pts
    30-50%, for pts <60 y.o.
  452. Cornerstone for tx of MDS
    Supportive care
  453. MDS prognosis
    • Ultimately fatal disease;
    • Infections or bleeding common causes of death
  454. Risk of transformation to leukemia depends on:
    Percentage of blasts in BM
  455. Etiology of multiple myeloma; assoc with:
    • Etiology unknown;
    • assoc w/pesticides, paper production, leather tanning, exposure to radiation from nukes
  456. Replacement of normal bone marrow by plasma cells leads to bone marrow failure
    Multiple myeloma
  457. Lytic lesions predisposing patients to bone pain, pathologic fractures, and hypercalcemia
    Multiple myeloma
  458. Multiple myeloma S/S
    • Fatigue/Anemia;
    • Bone pain (from lytic lesions: back and ribs);
    • Recurrent infxn;
    • Sp cord compression;
    • Unexplained fractures;
    • Kidney failure;
    • Hyperviscosity syndrome
  459. Multiple myeloma findings
    • Anemia;
    • Rouleaux;
    • M-spike on SPEP;
    • Bence-Jones proteins in Urine;
    • Hypercalcemia from bony dz;
    • Renal failure from light chain excretion
  460. Multiple myeloma classic triad:
    • Plasmacytosis (BM bx w/plasma cells > 5%);
    • Bone lytic lesions (on bone survey );
    • M-protein in serum and/or urine
  461. Important to differentiate btw multiple myeloma and:
    MGUS (monoclonal gammopathy of unknown significance)
  462. Multiple myeloma evaluation
    • SPEP (M-spike);
    • 24 hr urine (Bence-Jones pro);
    • Serum viscosity (may req plasma pheresis);
    • Metastatic bone survey (lytic lesions);
    • BM bx (inc. cytogenetics for q13- chromosome abnormality);
    • Beta-2 microglobulins
  463. Part of eval for multiple myeloma (if elevated = very poor outcome)
    Beta-2 microglobulins
  464. Thalidomide may be part of tx for:
    Myelofibrosis, MDS, multiple myeloma
  465. Multiple myeloma mgmt
    • Chemo;
    • Local radiation (pain ctrl);
    • Autologous BM txplt for LT survival (mortality rate of 40-50%); hypercalcemia tx (bisphosphonates)
  466. Multiple myeloma Prognosis:
    • Median survival w/ transplant = 7 yrs;
    • Median survival with chemo: 3 yrs
  467. MGUS prevalence
    • Present in 1% all adults, 3% over 70yr;
    • Progresses to multiple myeloma 25% of cases
  468. What MGUS looks like
    Usually, pts have monoclonal IgG spike <2.5g/dL, M-spike remains stable
  469. Malignancy of B lymphocytes
    Waldenström’s Macroglobulinemia
  470. Waldenström’s Macroglobulinemia causes overproduction of:
    Monoclonal macroglobulin (IgM antibody)
  471. Median age of onset is 64 yrs
    Waldenström’s
  472. Waldenström’s S/S
    • Present w/fatigue;
    • Hyperviscosity syndrome (nausea, vertigo, visual disturbances, mucosal or GI bleeding);
    • wt loss, HA, cold hypersensitivity, peripheral neuropathy, hepatomegaly, splenomegaly, engorged retinal veins
  473. Fatigue, cold hypersensitivity, peripheral neuropathy, engorged retinal veins may indicate:
    Waldenström’s Macroglobulinemia
  474. Hallmark of Waldenstrom:
    Monoclonal IgM spike in SPEP
  475. Waldenström’s Macroglobulinemia Findings
    • Anemia ;
    • Plasmacytic lymphocytes on BM bx;
    • Serum viscosity 1.4 to 1.8 x that of water;
    • Bone radiographs normal
  476. Waldenström’s is differentiated from MGUS by:
    Presence of bone marrow infiltration
  477. Waldenström’s Macroglobulinemia mgmt
    • If asymptomatic, follow expectantly;
    • Plasmapheresis for hyperviscosity syndrome;
    • Fludarabine & Rituximab preferred to alkylating agent tx;
    • BM txpt? ;
    • Median survival 3-5 yrs
  478. Most commonly occurring leukemia
    CLL
  479. CLL Mgmt
    • Observation; chemo;
    • BMT;
    • Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics);
    • Radiation (ctrl bulky adenopathy);
    • Surgery (for diagnostic)
  480. CLL prevalence
    Mainly disease of older people (>90% of cases >50yr); M:F = 2:1
  481. Characterized by clonal proliferation and accumulation of mature-appearing B lymphocytes (95 % of cases) in blood and lymphoid tissue; clonal malignancy of B lymphs
    CLL
  482. CLL S/S
    • 25 % asymptomatic at dx;
    • fatigue;
    • drenching night sweats;
    • lymphadenopathy (80%);
    • wt loss;
    • frequent/persistent infxn;
    • skin infxn/shingles
  483. CLL findings
    • Anemia & thrombocytopenia;
    • mature small lymphs & smudge cells;
    • Hepatomegaly;
    • Splenomegaly
  484. RAI Staging System used for:
    CLL
  485. CLL growth
    Tends to be slow growing and indolent
  486. Lymphocytosis, WBCs >20,000/µL = hallmark of:
    CLL
  487. RAI Stage 0
    • Inc WBC (leukocytosis blood & marrow);
    • >150 mos survival
  488. RAI Stage 1
    • Inc WBC & lymphadenopathy;
    • >101 mos survival
  489. RAI Stage 2
    • Inc WBC & Hepato/splenomegaly;
    • >71 mos survival
  490. RAI Stage 3
    • Inc WBC & Anemia (Hgb < 11g/dl);
    • >19 mos survival
  491. RAI Stage 4
    • Inc WBC & Thrombocytopenia;
    • >19 mos survival
  492. Blast crisis
    When blasts comprise >30% of BM cells
  493. CML will likely transform to:
    Acute disease
  494. Primarily disease of children
    ALL
  495. ?? % of ALL pts are children (usually 3 -7 yo)
    80%
  496. Accounts for 10-20% of acute adult leukemia
    ALL
  497. CML mgmt
    • Allo BMT;
    • chemo (Gleevec; Dasatinib, AMN107; Interferon)
  498. 3 phases of CML (defined by # blasts in marrow)
    Chronic, accelerated, and acute
  499. Slowly progressing dz: too many WBCs made in BM (esp myeloid cells)
    CML
  500. CML prevalence
    • Usually in young to middle age adults (medium age: 42);
    • CML accounts for 7-20% cases of leukemia
  501. Philadelphia chromosome present in ??% of CML cases
    >95% of cases (t9:22 translocation of DNA)
  502. _____ can trigger Philadelphia chromosome
    Exposure to radiation
  503. CML S/S
    • Fever (w/o infxn); bone pain;
    • LUQ pain (enlarged spleen);
    • night sweats;
    • bleeding & bruising;
    • petechiae;
    • fatigue ;
    • weakness
  504. CML findings
    • Median WBC = 170,000 at dx;
    • splenomegaly;
    • BM/PBS: Phil. Chr (bcr/abl fusion gene);
    • FISH for bcr/abl (quantitative);
    • RT-PCR for bcr/abl (qualitative);
    • thrombocytopenia (30%)
  505. CML tx goal:
    • Complete hemo & cytogenetic response;
    • Five yr survival = 52-63% ;
    • Median survival =6 yrs
  506. Type of leukemia = immature, abnormal cells in BM (>20%) and blood (>10%) & in liver, spleen, lymph nodes
    Acute Leukemia
  507. ALL prognosis
    • 80% of children will be cured with chemo;
    • 20-40% of adults will be cured
  508. ALL & AML S/S
    • The usual S/S plus meningitis;
    • Fever (abrupt onset with children);
    • petechiae;
    • anorexia
  509. ALL & AML labs
    • Pancytopenia;
    • hyperleukocytosis;
    • BM >20% blasts
  510. AML: median age at onset:
    65 yr
  511. AML: 5 year survival:
    10-30%
  512. Hyperleukocytosis
    Circulating blasts in peripheral blood (> 200,000/ mcl)
  513. ALL mgmt
    • Aggressive combo chemo [approx 2 yrs total (Cytoxan, daunorubicin, vincristine, prednisone)];
    • CNS prophylaxis (intrathecal chemo);
    • BMT??
  514. Chemo phases for ALL
    • Induction phase (4-6 wks);
    • Consolidation phase (several mos);
    • Maintenance phase (2-3 yrs)
  515. AML Etiology
    Poss: exposure to toxins (benzenes, radiation, chemotherapy)
  516. AML findings
    • Pancytopenia (& circulating blasts) (poss leukocytosis > 200,000);
    • BM >20% blasts;
    • Auer Rods;
    • high ESR;
    • hepatosplenomegaly
  517. AML mgmt
    • Chemo;
    • Induction & 3 consolidation treatments, in hospital;
    • Chemo = ARA-C with mitoxantrone, idarubicin, or daunorubicin;
    • BMT;
    • Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics)
  518. Hairy Cell leukemia prevalence
    • Median age of onset = mid 50s;
    • M:F = 5:1;
    • usually indolent, very responsive to tx
  519. Hairy Cell leukemia presentation
    • Fatigue, abd discomfort (markedly enlarged spleen);
    • persistent infxn;
  520. Hairy Cell leukemia tx
    • 2-CDA (2-chlorodeoxyadenosine), oral for 5-7 days (watch for drop in CBC counts);
    • 90% of pts complete remission
  521. Dz’s tx’d w/tumor lysis
    AML &CLL & NHL
  522. Hallmark of Hairy Cell leukemia
    • Pancytopenia;
    • “hairy” cells
  523. Hodgkin Dz
    • Nodular Sclerosing (80%);
    • LPHD;
    • Classical Hodgkin Dz;
    • Mixed cellularity
  524. Non-Hodgkin Dz
    • Follicular;
    • Burkitt;
    • Diffuse lg B-cell;
    • Marginal zone;
    • Cutaneous T-cell;
    • Anaplastic large cell
  525. Group of cancers w/enlarged lymphoid tissue, spleen, liver, & Reed-Sternberg cells
    Hodgkin Dz
  526. Hodgkin Dz prevalence
    • 7,900 new cases annually,
    • two peaks for age of onset: age 20-40 then after 50;
    • rare in kids <5;
    • more common in men 15-45
  527. _____ found in 40 – 50% of Hodgkin dz cases
    EBV
  528. Reed-Sternberg cells
    Hodgkin Dz
  529. Hodgkin Dz mechm of spread in pt
    Usually arises in a single area and spreads to contiguous nodes (“next-door dz”)
  530. Hodgkin Dz S/S
    • Painless cervical , supraclavicular, & mediastinal lymphadenopathy;
    • constitutional B symptoms (fever, drenching night sweats, wt loss);
    • SOB common with mediastinal mass
  531. Hodgkin Dz findings
    • Nodular sclerosis (esp young pt);
    • Mixed cellularity (esp in older pts);
    • prob infxs etiology in young pts (mononuc, x3 risk);
    • 5-10% = extranodal presentation (lung, liver, bone marrow);
    • Chest Xray: often mediastinal mass
  532. Hodgkin Dz diagnostics
    • CT chest, abdomen, pelvis;
    • PET scan;
    • BM bx
  533. Hodgkin Dz Stage I:
    one lymph node region
  534. Hodgkin Dz Stage II:
    two lymph node areas but only one side of the diaphragm
  535. Hodgkin Dz Stage III:
    nodal disease, both above and below the diaphragm
  536. Hodgkin Dz Stage IV:
    extranodal disease
  537. Hodgkin Dz mgmt: Stage I and IIa:
    local disease, radiation alone
  538. Hodgkin Dz mgmt: Stage IIb:
    Controversial, radiation +/- chemotherapy
  539. Hodgkin Dz mgmt: Stage III:
    nodal disease, both above and below the diaphragm, chemo +/-radiation (ABVD)
  540. Hodgkin Dz mgmt: Stage IV:
    Extranodal disease, chemotherapy
  541. Hodgkin Dz: Five year survival rate:
    > 80% (Will usually recur in 2 yrs if at all)
  542. Hodgkin Dz: if relapse:
    Consider high dose chemo followed by BMT
  543. Hodgkin Dz mgmt
    • Chemo: ABVD; give chemo q 2 weeks x 4-6 cycles;
    • Re-scan after 2-3 cycles to detn response;
    • Radiation follows chemotherapy;
    • Watch for toxicity/neutropenia (use CSFs); sperm banking?
  544. 5th most common malignancy in US
    NHL
  545. NHL prevalence
    • Approx. 50,000 new cases annually;
    • incidence higher in pts w/immunodeficiencies, h/o EBV, exposures to pesticides/solvents
  546. Dz: arise from cells in Lymphoid tissue (90% of cases are derived from B-lympocytes)
    NHL
  547. 3 Grades of NHL
    • Indolent (low grade, slow growing);
    • Intermediate (aggressive, mix of small to large cells);
    • High Grade (very aggressive)
  548. NHL S/S
    • Lymphadenopathy: persistent, painless, isolated or diffuse (retroperitoneum, mesentery, pelvis, extranodal – skin, GI tract);
    • B symptoms: fever, night sweats, weight loss, (intermediate and high-grade dz);
    • Abd pain, vomiting, bleeding, edema
  549. NHL findings
    • Normal CBC but poss anemia, thrombocytopenia, & leukopenia; occ lymphoma cells on diff;
    • bulky lymphadenopathy (poss cause jaundice, hydronephrosis, SVC syndrome, bowel obstruction, wasting)
  550. NHL staging
    • CT chest, abdomen, pelvis;
    • PET scan??;
    • Unilateral or bilateral BM bx;
    • LDH (tumor marker);
    • LP if CNS dz is suspected
  551. NHL mgmt
    • Range of options from watch-and-wait to intensive chemo, with/without radiation;
    • Tumor lysis prophylaxis (allopurinol, 300 mg/day, hydration, diuretics);
    • BMT
  552. NHL tx depends on:
    specific type and grade of NHL
  553. Role of surgery in NHL
    Diagnostic
  554. Mgmt of Low Grade NHL
    • Watch & wait (“dynamic observation”) average of 6 yrs after dx;
    • Rituximab (monoclonal antibody targets B-cells SAg CD20 cells), 1x/wk for 4 weeks;
    • R-CHOP (rituximab plus cytoxan, adriamycin, vincristine, prednisone);
    • CHOP
  555. Mgmt of Intermediate Grade NHL
    • Rituxan-CHOP;
    • R-ICE; B
    • exxar (radiolabeled I-131);
    • Zevalin[ radioimmunotherapy for NHL (radio-labelled Rituxan)]
  556. Mgmt of High Grade Lymphoma ((Large B-Cell Lymphoma)
    • R-CHOP;
    • R-ICE;
    • many lymphomas tx’d w/specific tx’s (Burkett lymphoma, post transplant lymphoproliferative disorder);
    • Lymphoblastic lymphomas are treated with regimens similar for T-cell ALL
  557. Lymphoblastic lymphomas are treated with:
    Regimens similar for T-cell ALL
  558. Oncologic emergencies
    • Febrile Neutropenia;
    • SVC Syndrome (superior vena cava syndrome);
    • Tumor Lysis Syndrome; Hypercalcemia;
    • Cord compression (myelomas)
  559. Can affect T or B lymphocytes
    ALL
  560. Type of HL that accounts for 80% of HL cases
    Nodular sclerosing HL
  561. Bulky lymphadenopathy seen in:
    CLL; NHL
  562. Only curative tx for MDS
    Allogeneic BMT
  563. Smudge cells
    CLL
  564. Serum viscosity 1.4-1.8 xH2O visc
    Waldenström’s
  565. Next-door dz
    HL
  566. Pancytopenia
    ALL, AML, hairy cell
  567. Giant plts & teardrop poik
    Myelofibrosis
  568. Drenching night sweats
    CLL, Hodgkin’s
  569. B-symptoms
    fever, wt loss, drenching night sweats (HL, NHL)
  570. Lymphadenopathy (in 80%)
    CLL
  571. Skin infxn/shingles
    CLL
  572. RAI staging is for
    CLL
  573. Lytic lesions
    multi myeloma
  574. Smudge cells
    CLL
  575. Serum viscosity 1.4-1.8 xH2O visc
    Waldenström’s
  576. Next-door dz
    HL
  577. Pancytopenia
    ALL, AML, hairy cell
  578. Giant plts & teardrop poik
    Myelofibrosis
  579. Drenching night sweats
    CLL, Hodgkin’s
  580. B-symptoms
    fever, wt loss, drenching night sweats (HL, NHL)
  581. Lymphadenopathy (in 80%)
    CLL
  582. Skin infxn/shingles
    CLL
  583. RAI staging is for
    CLL
  584. Lytic lesions
    MM
  585. Auer rods
    AML
  586. Dohle bodies
    • blue patch near edge;
    • PMN;
    • infxn
  587. Toxic gran
    • purple/blue-black; PMN;
    • severe infxn/toxic
  588. Hyperseg
    • 6or more/3% w/5 or more;
    • PMN;
    • megaloblastic anemia, B12-folate def
  589. Vacuoles
    • Monos/PMN;
    • poss sepsis
  590. BM aspirate
    iliac crest
  591. LAP
    • leukocyte alkaline phosphatase;
    • Low in CML;
    • High in leukemoid reactions
  592. Meningitis
    AML/ALL
  593. Fried egg
    hairy cell
  594. Hypercellular bone marrow with reticulin or collagen fibrosis
    myelofibrosis
  595. Preleukemia
    MDS
  596. Immunoproliferative dz
    • Waldenström’s;
    • Multiple Myeloma;
    • MGUS
  597. Plts in leukemias
    • Increased (acute);
    • normal (chronic)
  598. Immunologic phenotypes = Common; Early B lineage; T cell
    ALL
  599. M0-M7 phenotypes
    AML
  600. Coexpression of CD19, CD5
    CLL
  601. Hypogammaglobulinemia
    CLL
  602. Anemia seen in leukemias
    MM, hairy cell (NOT seen in CML)
  603. Isolated lymphocytosis
    CLL
  604. Lymphocyte pleomorphism; heterophile Ab
    EBV
  605. Clustered bacteria in vacuoles in WBCs; low plts; leukopenia w/left shift; Rising Ab immunofluorescence titer
    Ehrlichiosis
  606. Histoplasmosis CBC
    • Organism in neutrophils & monocytes on Wright-Giemsa PBS;
    • in macrophages on bone marrow smear
  607. Engorged retinal veins
    P vera; Waldenström
  608. Normocytic
    anemia w/ normal sized RBC
  609. Normochromic
    hemoglobin # normal
  610. RDW
    • Red cell distribution width;
    • blood test measuring RBC size
  611. Anisocytosis
    anemia w/ various-sized RBC
  612. Poikilocytosis
    anemia w/ various-shaped
  613. Hematopoiesis
    formation of blood or blood cells
  614. Erythropoiesis
    production of RBC
  615. Granulopoiesis
    production of granulocyte
  616. Erythropoietin
    glycoprotein hormone by kidney stimulating BM for RBC production
  617. Blood Indices
    MCV. MCH. MCHC
  618. MCV
    mean corpuscular volume (size)
  619. MCH
    mean corpuscular hemoglobin, hbg/RBC
  620. MCHC
    mean corpuscular hbg concentration; hgb/hct
  621. Blood
    • 5 liter in adult human,
    • 6 - 8% total body wt.,
    • should only exist in circulatory system
  622. Capillaries
    microscopic tubules, not in cornea and cartilage

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