Heme PANCE

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Heme PANCE
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2012-08-09 10:45:49
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HEME PANCE
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  1. M/C cause of anemia in the world
    IDA
  2. m/c cause of IDA (in US)
    blood loss
  3. What is the definition of severe IDA?
    Hct < 25%
  4. PBS of IDA
    • Nl initially
    • Later hypochromic microcytic red cells, anisocytosis, and poikilocytosis
  5. Hgb H dz
    only one alpha chain present
  6. Cooley anemia
    B thall major
  7. When is Cooley's anemia dx?
     problems begin at 4-6 months, when the switch from fetal Hgb to adult Hgb
  8. How to differentiate b/w thalassemia and IDA
    Serum iron and ferritin levels are nl
  9. How do you dx thalassemia?
    Hgb elcectrophesis
  10. How do you tx thalassemia?
    • folic acid supplements, avoid iron suppleemnts and oxidative meds
    • B major: transfusions
  11. How do you avoid hemosiderosis in thalassemia tx?
    Deferoxamine
  12. What type of anemia is sideroblastic anemia?
    hypochromic
  13. How do you dx sideroblastic anemia?
    Prussian Blue staining
  14. What are causes of sideroblastic anemia?
    myelodysplasia, chronic alcoholism, and lead poisoning
  15. m/c cause of normochronmic normocytic anemias
    Te cell meidate autoimmune suppression of hematopoesis
  16. S/Sx of aplastic anemia
    weakness, fatigue, vulnerability to infection, pallor, purpura, and petechiae
  17. what is the hallmark of aplastic anemia?
    pancytopenia
  18. What type of anemia is aplastic anemia?
    normochromic normocytic
  19. m/c cause of folic acid deficiency
    poor dietary intake
  20. What meds are folic acid antagonists?
    Phenytoin, Bactrim, sulfasalazine
  21. What is the daily requirement of folic acid?
    50-100 mg
  22. What disease states increase folic acid requirements?
    Pregnancy, hemolytic anemias, and exfoliative skin dz
  23. What is pathognominc for folic acid deficiency?
    Macro-ovalocytes and hypersegmented PMNs
  24. What does a PBS of folic acid deficiency look like?
    macroovalocytes, hypersegmented PMN, Howell-Jolly bodies
  25. What are Howell-Joly bodies?
    nuclear DNA remnants
  26. How do you manage folic acid?
    1 mg/day
  27. what is the m/c cause of B12 anemia
    pernicios anemia
  28. Where does absorption of B12 occur?
    terminal ileum, storage in the liver
  29. S/Sx of B12 deficiency
    • glossitis, pale icterus, vague GI sx
    • neur: stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, clumsiness, dementia, and ataxia
  30. PBS of B12 deficiency
    anisocytosis, poikilocytosis, macro-ovalocytosis, and hypersegmented neutrophils
  31. Lab findings of B12 deficiency
    Reduced reticulocyte count, elevated LDH and indirect bilirubin, low B12
  32. Tx of b12
    lifelong Im injections for pernicious anemia, daily oral cobalmin in high doses
  33. When are B12 neurological sx reversible?
    Treated within 6 months
  34. Name intrinsic hemolytic anemias
    hereditary spherocytosis and elliptocytosis, G6PD, methemoglobinemia, SCA
  35. What are extrinsic causes of hemolytic anemia?
    autoimmune and lymphoproliferative diseases, drug toxicity, TTP, HUS, DIC, valvular hemolysis, metastatic adenocarcinoma, vasculitides, infections, hypersplenism, burns
  36. Presentation of hemolytic anemias
    jaundice, gallstones, pallor, and sx
  37. Infection with what pathogen can cause transient aplastic crisis?
    Parvovirus
  38. What is the hallmark of hemolytic anemia?
    elevated reticulocyte count in presence of falling or stable HCT
  39. PBS of hemolytic anemia
    immature red cells, nucleated red cells, nucleated red cells, or morphologic changes
  40. What are lab findings in hemolytic anemia?
    transient hemoglobinemia, elevated unconjugated bili, serum LDH high (microangiopathic hemolysis)
  41. Genetics of SCA
    autosomal recessive
  42. What is pathophys of SCA?
    RBCs containing primarily HGB S sickle under deoxygenated conditions
  43. What increases sickling in SCA?
    red cell dehydration, acidosis, hypoxemia, high altitudes
  44. People with SCA are at risk for what?
    cholelithiasis, splenomegaly, poorly healing ulcers, infection with encapsulated organisms, strokes, priapism, retinopathies, osteomyelities, avascular necrosis
  45. How do you dx SCA?
    HGB electrophoresis, PBS
  46. PBS of SCA
    sickled cells and target cells, nucleated RBCs, HJ bodes
  47. Lab findings of SCA
    • elevated tic count
    • Elevated wbC count
    • thrombocytosis may be present
    • indirect bili may be elevated
  48. How do you tx SCA?
    • Analgesics, fluids, oxygen
    • transfusions for vaso-occlusive crises
    • Pneumococcal vaccine q10 years, folate supplementation
  49. Genetics of G6PD
    x-linked recessive disorder commonly seen in American black males and some mediterreanean populations
  50. List oxidative drugs
    ASA, dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin)
  51. Lab findings of G6PD
    • tic count and serum indirect bili increase during hemolytic episodes
    • PBS reveal bite cells and Heinz bodies
    • G6PD levels low
  52. PBS of G6PD
    bite cells and Heinz bodies
  53. Tx of G6PD
    • self-limited as RBCs are replaced
    • avoid oxidative drugs
  54. What is PCV?
    • slowly progressive bone marrow disorder characterized by increased numbers of RBCs and increased total blood volume;
    • unregulated expansion of RBC causes hyperviscosity, which leads to decreased cerebral blood flow
  55. What is dx of PCV?
    presence of JAK2 mutation
  56. m/c cause of mortality/morbidity in PCV
    thrombosis
  57. epidemiology of PCV
    M/F, median age of presentation 60%
  58. Avg survival time of someone with PCV
    11-15 years
  59. PCV can convert to what?
    myelofibrosis or CML, rarely to AML
  60. What are dx criteria for PCV?
    splenomegaly, nl arterial O2 sat, elevated RBC mass
  61. Sx of PCV
    sx of increased blood viscosity (HA, dizziness, fullness in head and face, weakness, fatigue, tinnitus, blurred vision); burning, pain, redness of extremities, stroke
  62. Pruritus after bathing
    PCV
  63. What are common comorbidities with PCV?
    • epistaxis (may be presenting sx)
    • hPUD
    • plethora, systolic HTN, engorged retinal veins, splenomegaly
  64. Lab findings in PCV
    • HCT > 54% males, 51% females
    • PBS shows neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts
    • nl RBC morphology
    • hypercellular bone marrow in all cell lines
    • hyperuricemia
  65. PCV PBS
    neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts
  66. How do you tx PCV?
    • phlebotomy is tx of choice
    • myelosuppressive therapy with hydroxyurea may be indicated; anagrelide may be added or substituted
    • low dose ASA reduces risk of thrombosis without increasing the risk of bleeding
  67. what is leukemia?
    diseases characterized by unrestrained growth of leukocytes and leukocyte precursors in the tissues
  68. M/c childhood leukemia
    ALL
  69. age range of ALL
    3-7 yo
  70. Age range of AML
    avg age of onset 60 yo
  71. Clinical features of acute leukemias
    • gingival bleeding, epistaxis, menorrhagia; DIC is less common
    • fatigue, abrupt onset of fever, lethargy, HA, and bone and or jt pain (esp in sternum, tibia, and femur)
  72. what is the hallmark of acute leukemia?
    pancytopenia with circulating blasts; blasts make up at least 20% of nucleated cells in bone marrow
  73. What are lab findings in acute leukemia?
    • hyperuricemia
    • pancytopenia with circulating blasts
    • Auer rods in AML
    • high WBC
  74. Auer rods
    AML
  75. Leukmia with mediastinal mass
    ALL
  76. Presence of Philadephia chromosome is unfavorable
    ALL
  77. What is CLL?
    clonal malignancy of B lypmhocytes
  78. What is the m/c leukemia?
    CLL
  79. What is the m/c form of CLL?
    B cell form
  80. What is the rai system?
    prognostic staging for CLL
  81. Epidemiology of CML
    young to middle aged adults (mediat age is 55 years)
  82. Three phases of CML
    chronic, accelerated, and acute (aka blast crisis, >30% blasts)
  83. Sx of CML
    • fatigue, anorexia, weight loss, low-grade fever, excessive sweating
    • abdominal fullness caused by splenomegaly
    • runs a mild course until blast phase
  84. clinical features of CLL
    • peripheral lymphocytosis and lymphotic invasion of bone marrow, liver, spleen, and LNs
    • recurrent infections, HSM
  85. Richter's syndrome
    a type of CLL, where an isolated node transforms into aggressive, large cell lymphoma
  86. What is the hallmark of CLL?
    isolated lymphocytosis (leukocytosis > 20,000 cells)
  87. What is the hallmark of CML?
    leukocytosis, with meadian WBC count of 150,000
  88. BCR-ABL
    gene used to idenitfy chronic leukemia
  89. CML PBS
    anemia and thrombocytosis
  90. CLL PBS
    mature small lymphocytes, smudge cells
  91. Smudge cells
    CLL
  92. Tx of CML
    • Gleevec, dastinib or nilonib in cases of Gleevec intolerance
    • allogenic bone marrow transplant may bt eh initial tx and is the only therapy proven to be curative
  93. Tx of Acute leukemias
    • chemotherapy, consolidation therapy
    • bone marrow transplant if poor reponse to tx
  94. Tx of CLL
    pallaitive once dz is advanced
  95. Hodgkin's disease
    group of cancers characterized by enlargement of lymphoid tissue, spleen, and liver and the presence of reed-sternberg cells
  96. Reed Sternberg cells
    Hodgkin's Disease
  97. Virus associated with Hodgkin's Disease
    EBV
  98. Age range Hodgkin's disease
    15-45 yo, peaking in 20s, and again after 50; rare under 5 yo
  99. Clinimcal features of Hodgkin's disease
    painless cervical, supraclavicular, and mediastinal lymphadenopathy; pain in affected node after ingestion of alcohol
  100. Stage A Hodgkins
    lack of constituational sx
  101. What indicated a poorer prognosis in HD?
    presence of constitutional sx
  102. Ann Arbor
    Staging system for Hodgkins and NHL
  103. Tx of HD
    • combo chemo
    • radiation therapy nitial tx of choice for pts with low risk stage IA and IIA dz
    • most other pts receive adriamycin, bleomycin, vinblastine, and dacarbazine chemo (ABVD)
  104. NHL
    group of malignancies that arise from lymphocytes, usually from B lymphocites
  105. epidemiology of NHL
    Peak incidence between 20 and 40 yo, lymphomas divided into clinically indolent and addgressive groups
  106. Clinical features of NHL
    diffuse or isolated, painless, persistant lymphadenopathy is the m/c presentation, extralymphatic presentation, some constitutaionl sx
  107. m/c presentation of NHL
    diffuse or isolated, painless, persistant lymphadenopathy
  108. Extralymphatic presentations of NHL
    GI tract, skin, bone, and bone marrow
  109. M/c presentation of burkitt's lymphoma
    abdominal fullness
  110. What is used as a prognostic marker for NHL?
    Serum LDH
  111. Tx of NHL
    • some spontaneous remission
    • single involved node: radiation
    • low grade lymphoma: rituximab with or without chemo
    • aggressive: Low grade lymphoma=allogenic transplant
    • Intermediate or high grade lymphomas: chemo and autlogous stem cel transplant
  112. Multiple myeloma
    • malignancy of plasma cells, possibly caused by HPV
    • replacement of bone marrow leads to failure, bone destruction leads to pain, OP, lytic lesions, hypercalcemia, and pathologic features
  113. Why are MM pts prone to recurrent infections?
    Neutropenia and failure of Ab production in reponse to antigen challenge
  114. median age of dx of MM
    65 yo
  115. M/c presenting complaints of MM
    • anemia, bone pain (particularly in low back or ribs, and infection)
    • also renal failure, spinal cord comperssion, and hyperviscosity syndrome
  116. Rouleax formation
    MM
  117. Monoclonal spike on protein electrophoresis
    MM
  118. lytic lesions on xray with generalized Op
    MM
  119. Tx of MM
    lenalidomide, dexamethasone, and doxorubicin; with bisphosphonates as adjuncts
  120. What does thrombin clotting time measure?
    rate of conversion between fibrinogen and fibrin in the presence of thrombin
  121. m/c cause of abnl bleeding
    TCP
  122. Causes of TCP
    impaired production, increased destruction, splenic sequestration, or dilution
  123. ITP
    self limited, auto-immune disorder found commonly in children of both sexes and is associated with a preceeding viral URI
  124. Associated with URI
    ITP
  125. Sx of acute ITP
    abrupt appearance of petechaie, purpura, and hemorrhagic bullae
  126. Chronic ITP presentation
    petechiae on skin and mucous membranes
  127. What drug m/c causes ITP type reaction?
    Heparin
  128. what other drugs can cause ITP?
    sufulonamides, quinine, thiazides, cimetidine, and gold
  129. Lab findings of acute ITP
    • decreasd plalets (b/w 10-20k), eosinophilia, and mild lymphocytosis
    • mild anemia, nl coag
  130. lab findings of chornic ITP
    • plts b/w 25-75k
    • mild anemia, nl coag
  131. Tx of acute ITP
    usually resolves spontaneously, some pts require corticosteroids or splenectomy
  132. chronic ITP tx
    • high dose prednisone
    • If tx fails: IVIG, danazol, immunosuppresive therapy, stem cell transplant
  133. Three types of plt consumption syndromes
    TTP, HUS, and DIC
  134. Epidemiology of TTP
    20-50 yo, m/c in women than men, common in HIV
  135. What can precipitate TTP?
    Estrogen use, pregnancy, drugs such as quinine and ticlopidine
  136. What is TTP?
    Abs against ADAMTS13 enzyme, which breaks down large multimers of VWF. therefore an increase in VWF leads to an increase in clotting at sites of vascular injury
  137. What is DIC?
    generalized hemorrhages in pts with severe underlying systemic illness such as sepsis, tissue injury, obstetric complications, cancer, and severe transfusion reactions
  138. What is the TTP pentad? What are other sx?
    • purpura, microangiopathic hemolytic anemia, fever, abnl neurologic signs, renal failure
    • petechiae, apllor, abdominal pain, possibly pancreatitis
  139. What is HUS?
    Child version of TTP without neurologic findings, but with more kidney problems
  140. Who does HUS affect?
    <10 yo, particularly after infection with E coli, shigella, salmonella, and various viruses
  141. What are s/sx of DIC?
    skin and mucous membrane bleeding (particularly at puncture/wound sites), shock, thrombosis
  142. What are lab findings of TTP?
    • anemia, red cell fragmentation (Schistocytes), nl leukocytes, polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in TTP), negative Coombs
    • High LDH
    • High indirect bili
    • nl coag
    • renal insufficienc
  143. What are lab findings of HUS?
    • anemia, red cell fragmentation (Schistocytes), nl leukocytes,
    • polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in
    • TTP), negative Coombs
    • High LDH
    • High indirect bili
    • nl coag
    • renal insufficienc
  144. What are lab findings in DIC?
    • Evidence of coagulopathy (hypofibrinogenmia, elevated fibrin degradation products)
    • TCP
    • prolonged PT
    • Absent ADAMTS13
    • Microangiopathic hemolytic anemia with schistocytes
  145. shisctocytes
    fragmented RBCs
  146. Tx of TTP
    • large volume plasmapheresis
    • prednisone and antiplatelt agents
  147. Tx of HUS
    • conservative management
    • fluids and management of electrolyte imbalance
    • in adults, plasmapheresis
  148. DIC tx
    • tx underlying cause
    • component blood transfusions are important; role of heparin is controversial
  149. m/c cause of acquired plt dysfunction
    NSAIDS, ASA
  150. Genetics of VWD
    autosomal dominant
  151. m/c congenital coagulopathy
    vWD
  152. Clinical features of vWD
    • bleeding in nasal, sinus, vaginal, and GI mucous membranes
    • rare bleeding into joint spaces
  153. lab findings in vWD
    • nl pt/ptt, prolonged bleeding time
    • low vwf
  154. How do you tx VWD?
    • vasopressin for type I
    • factor VIII concentrates are preferred if factor replacement is necessary
  155. Factor VIII deficiency
    Hemophilia A
  156. What is hemophilia a?
    a hereditary disease caused by excessively prolonged coagulation time
  157. genetics of hemophilia A
    x linked recessive
  158. most severe bleeding disorder
    hem a
  159. Clinical features of Hemophilia A
    • Spontaneous hemorrhagic episodes with hemearthroses, epistaxis, intracranial bleeding, hematemesies, melena, microscpoic hematuria, bleeding into soft tissue and gingiva
    • excessive bleeding following trauma/surgery
  160. Lab findings in Hem A
    • Prolonged PTT
    • PT, bleeding time, fibrinogen level, plts nl
    • reduced factor 8, vwf nl
  161. How do you tx Hem A?
    • infusion of heat treater or recombinant factor 8
    • desmopressin may elevated levels in mild to mod dz
    • avoid ASA, use celecoxib or opiods for pain
  162. Factor 9 deficiency
    Hem B
  163. Christmas disease
    Hem B
  164. genetics of Hem B
    X linked recessive disorder
  165. Favtor XI deficiency
    found in Ashkenazi Jews
  166. Most common acquired coagulopathies
    Vit K
  167. What are causes of Vit K deficiencies?
    • poor diet
    • liver failure
    • malabsorption
    • malnutrition
    • use of some drugs
  168. Labs in Vit K dependent factor deficiencies
    • PT prolonged, PTT may be prolonged
    • Fibrinogen, thrombin time, plt count are nl
    • liver enzymes may be elevated
  169. Tx of Vit K dependent factor deficiencies
    • Tx underlying cause
    • Vit K (PO or IV)
    • tx hemrorhage with FFP
  170. Vit K dependent factors
    2, 7, 9, 10
  171. Trousseau's Syndrome
    Acquired hypercoaguable states associated with malignancy

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