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  1. Describe the Intrinsic Coagulation Pathway
    Contact Activation Pathway

    High Molecular Weight Kininogen + Factor XII starts cascade with collagen and prokallikrein
  2. Describe the Extrinsic Coagulation Pathway
    This is called the Tissue Factor Pathway

    Thromboplastin (aka Tissue Factor) activates Factor VII and X.
  3. What is the rate limiting factor in the clotting cascade?
    Factor X
  4. What are the two accelerating factors in the clotting cascade?
    Factor V and VIII
  5. What two proteins inhibit the accelerating factors in the clotting cascade?
    Protein C and S (thrombotic)
  6. Describe the role of HMWK in the coagulation cascade.
    It activates bradykinin which stimulates the kinin pathway:

    increased vasodilation, permeability and pain
  7. Hemophilia A is a defect in what factor? Hemophilia B?
    • A - 8 (a-ate)
    • B - 9 (benign)
  8. Vitamin K deficiency results in the decreased synthesis of what factors?
    DISCO 1972

    Factors 2, 7, 9 and 10, Proteins C and S
  9. Antithrombin (an anticoagulant) inhbits what factors? What drug activates antithrombin?
    Inhibits: thrombin (increases clotting), 2,7,9,10,11 and 12

    Activated by heparin
  10. What enzyme activates Vitamin K to allow creation of coagulation cascade components? What drug inhibits this enzyme?
    Epoxide reductase

    Warfarin
  11. What counter-intuitive side effect can be seen with Warfarin?
    Transient hypercoagulation at first administration.

    Proteins C and S are inhibited, but they usually stop accelerating factors V and VIII, so they are temporarily unchecked.
  12. tPA is used clinically as a thrombolytic. How does it work?
    Plasminogen is turned into plasmin by tPA (tissue plasminogen activator) which cleaves fibrin mesh.
  13. What does PT test for? What does PTT test for?
    PT - tests extrinsic pathway.

    PTT - tests intrinsic pathway
  14. In Hemophilia A or B, what test, PT or PTT, would be increased?
    PTT - checking for factors 8 and 9 which are found only in intrinsic pathway.
  15. Which tests are elevated in Vitamin K deficiency? PT or PTT?
    Both - DISCO1972 describes factors in both pathways.
  16. Babies get a shot of this vitamin in the hospital because gut flora haven't yet developed to produce it.
    Vitamin K
  17. If you have a disorder of the platelets only, what test would you expect to see elevated?
    Coagulation factors are fine, so you only get an increase in bleeding time (BT) and PT, PTT are normal.
  18. Factor V Leiden disease - Pathogenesis and Symptoms
    Mutated Factor V = super fast clotting

    Hypercoagulability
  19. What is the most common cause of hereditary hypercoagulability?
    Factor V Leiden
  20. What do you see with a prothrombin gene mutation?
    venous clots
  21. What do you see with an antithrombin deficiency?
    resistance to heparin (which activates antithrombin, so if there isn't any in the blood - can't do its work)

    Hypercoag
  22. What do you see in a Protein C or S deficiency?
    decreased ability to inactivate factors V and VIII - hypercoag. increased risk of thrombotic skin necrosis after administration of Warfarin - can't shut off clotting cascade!
  23. Heparin - MOA, Clin Use, Toxicity
    MOA: activates antithrombin

    Clin Use: Pulmonary Embolism, Stroke, Acute Coronary Syndrome, MI, DVT - immediate anticoagulation

    Toxicity: HIT, Bleeding, Osteoporosis
  24. This anticoagulant is safe for pregnant women.
    Heparin
  25. LMW Heparin: MOA, Clin Use, Tox:
    MOA: acts on Xa instead of antithrombin only. Longer T1/2

    Clin Use: subcutaneous administration, less monitoring

    Tox: Not easily reversible
  26. What drug can you give to reverse too much Heparin?
    protamine sulfate
  27. Heparin Induced Thrombocytopenia - - Pathogenesis and Symptoms
    Heparin binds Factor 4 - this starts immune response and attacks platelets.

    Thrombocytopenia and hypercoagulable state
  28. What drug do you give to someone with HIT instead of Heparin? What is its MOA?
    Lepirudin - inhibits thrombin directly
  29. Warfarin - MOA, Clin Use, Tox
    MOA: inhibits epoxide reductase / Vitamin K

    Clin Use: Chronic anticoagulation (DVT prophylaxis), A Fib

    Tox: Teratogenic, Bleeding, Necrosis,
  30. What values should you test regularly when your patient is on Warfarin?
    INR and PT
  31. Compare Warfarin to Heparin: Structure, Route of Administration, Site of Action, Onset of Action, MOA, Treatment of Overdose, Monitoring and Placental cross
    • Structure: H - large, W - small
    • ROA: H - IV/SC, W - oral
    • SOA: H - blood, W - liver
    • OOA: H - rapid, W - slow (limited by factor T1/2's)
    • MOA: H - activates antithrombin, W - inhibits epoxide reductase
    • OD Tx: H - Protamine Sulfate, W - IV Vit K and Fresh Frozen Plasma
    • Monitor: H - PTT, W - PT/INR
    • Placental Cross: H - No (safe for preg), W - yes (teratogenic)
  32. Name 4 thrombolytic agents and their MOA:
    • Streptokinase
    • Urokinase
    • tPA (alteplase)
    • APSAC (anistreplase)

    MOA: converts plasminogen to plasmin - cleaves thrombin or fibrin clots.
  33. What do you see in terms of tests when tPA is administered?
    Increased PTT and PT
  34. What is the clinical use for thrombolytics?
    Early MI, Early Stroke
  35. What can you use to reverse bleeding from thrombolytics?
    Aminocaproic Acid
  36. Does enoxaparin (LMWH) need to be monitored?
    No
  37. What is the treatment for heparin induced thrombocytopenia?
    Hirudin (Lepirudin) drugs and protamine sulfate
  38. Where does fetal erythropoiesis occur?
    • Yolk Sac - 8weeks
    • Liver - 30 weeks
    • Spleen - 28 weeks
    • Bone marrow - >28 weeks
  39. How does fetal hemoglobin differ from adult hemoglobin?
    • Fetal - 2 a chains, 2 y chains - higher affinity for O2
    • Adult - 2a, 2b chains
  40. Erythrocyte: shape, energy source, survival time
    • Biconcave
    • Glucose
    • 120 days
  41. What is the protein responsible for the flexibility of RBC's?
    Spectrin
  42. What word describes RBC's of:

    1) varying size
    2) varying shape
    3) immature
    • Anisocytosis - size
    • Poikilocytosis - shape
    • Reticulocyte - immature
  43. What kind of hypersensitivity reaction happens if you transfuse the wrong blood type?
    Type II
  44. What blood type is considered the universal donor? Universal recipient?
    • donor - O
    • recipient - AB
  45. Describe the 4 steps of platelet plug formation
    • 1. Injury - vWF binds collagen
    • 2. Adhesion - platelets bind vWF and release ADP and Ca2+
    • 3. Activation - ADP induces GpIIb/IIIa expression on platelet
    • 4. Aggregation - Fibrinogen binds GpIIb/IIIa and links platelets.

  46. Cell Type and Associated Path:
    Acanthocyte (spur cell)

    • Liver Disease
    • Abetalipoproteinemia


  47. Cell Type and Associated Path:
    Basophillic Stippling

    "Baste the Ox TAIL"

    • Thalassemia
    • Anemia
    • Iron Deficiency
    • Lead Poisoning


  48. Cell Type and Associated Path:
    Bite Cell

    • G6PD deficiency
    • -> can't go through HMP shunt, causing oxidative damage resulting in Heinz Bodies which are chewed on by macrophages in the spleen


  49. Cell Type and Associated Path:
    Macroovalocyte

    Megaloblastic Anemia


  50. Cell Type and Associated Path:
    Ringed Sideroblast

    • Sideoblastic Anemia
    • Lead Poisoning


  51. Cell Type and Associated Path:
    Schistocyte - Helmet Cell

    DIC, TTP/HUS, Traumatic Hemolysis

    cells are forced through fibrin areas and get chopped up


  52. Cell Type and Associated Path:
    Sickle Cell

    Sickle Cell Anemia


  53. Cell Type and Associated Path:
    Spherocyte

    Hereditary Spherocytosis, Autoimmune Hemolysis


  54. Cell Type and Associated Path:
    Teardrop cell

    Myelofibrosis


  55. Cell Type and Associated Path:
    Target Cell

    HALT!

    • HbC disease
    • Asplenia
    • Liver Disease
    • Thalassemia


  56. Cell Type and Associated Path:
    Heinz Bodies

    iron oxidation

    seen in : a-thalassemia, G6PD deficiency


  57. Cell Type and Associated Path:
    Howell-Jolly Body

    Functional Apslenia - Sickle cell, splenic trauma, TTP/HUS
  58. Name the 3 different types of Polycythemia
    • Appropriate Absolute
    • Inappropriate Absolute
    • Polycythemia Vera
  59. Which type of polycythemia is associated with Lung disease, high altitude and congenital heart disease?
    appropriate absolute - incresaed RBC mass decreased O2 saturation
  60. Which type of polycythemia is associated with RCC, HCC, Wilms tumor and hydronephrosis?
    inappropriate absolute - due to neoplastic ectopic erythropoietin
  61. What is the pathogenesis of polycythemia vera?
    Monocolonal proliferation of RBC's
  62. Lead Poisoning: Affected enzyme and presenting symptoms and treatment
    Ferrochelatase and ALA dehydratase

    microcytic anemia, GI and kidney disease

    • Kids: mental deterioration
    • Adults: headache, memory loss

    Tx: Dimercaperol, EDTA, Succinyl
  63. Acute Intermittent Porphyria: Affected enzyme, presenting symptoms and treatment
    Porphobilinogen deaminase

    • Acute abdomen, acute neuro pain
    • Dark red wine urine, psych disturbances

    Tx: glucose and heme
  64. Porphyria Cutanea Tarda: Affected enzyme and presenting symptoms
    Uroporphyrinogen decarboxylase

    Homeless Guy!

    skin blister, photosensitivity, tea colored urine, excessive hair, related to alcohol, Hep C
  65. Which is the most common porphyria?
    Porphyria cutanea tarda
  66. What is the rate limiting step in heme synthesis?
    aminolevulinic acid synthase
  67. Name the 9 different types of Hemoglobin
    • HbA
    • HbA2
    • HbA1c
    • HbF
    • HbGower
    • HbS
    • HbC
    • HbBarts
    • HbH
  68. Which hemoglobin is the most common?
    HbA
  69. Which hemoglobins are associated with severe a-thalassemia?
    HbBarts and HbH
  70. Which hemoglobin is associated with fetal hb? How is it different?
    HbF

    two gamma chains instead of beta chains (in adults)
  71. What is substituted in HbS in Sickle Cell?
    Valine in B chain
  72. What is substituted in HbC in HbC Disease?
    Lysine in B chain
  73. Name the 5 microcytic anemias
    TAILS again, like in Basophillic stippling

    • Iron deficiency
    • ACD
    • Thalassemias
    • Lead Poisoning
    • Sideroblastic Anemia
  74. Iron Deficiency Anemia: Disorder and Findings

    Associated Disease?
    Chronic bleeding, malnutrition, decreased heme synthesis

    Microcytosis and hypochromia

    Plummer Vinson syndrome (DIG - Dysphagia, Iron Deficiency, Glossitis)
  75. A-thalassemia: Disorder and Findings
    defect in a-globulin chain

    Africans and Asians

    • 4 gene deletion - HbBarts = hydrops fetalis
    • 3 gene deletion - HbH disease
  76. B-thalassemia: Disorder and Findings
    point mutation -> decreased B globin

    Mediterranean people

    • Minor: asymptomatic
    • Major: chipmunk facies, crew cut on skull. Severe anemia, requires transfusions

    Major blood smear findings: weird shapes and sizes of RBC's, target cells, helmet cells, hypochromia
  77. Lead Poisoning: Disorder and Findings
    Lead inhibits ferrochelatase and ALA dehydratase


    • "LEAD BullShit"
    • Lead lines on long bones and gingiva
    • Encephalopathy
    • Abdominal Colic
    • Drops - foot, wrist
    • Basophillic stippling
    • Sideroblastic Anemia

    Tx: Dimercaprol, EDTA, Succimer
  78. Where are ringed sideroblasts found?
    In the bone marrow
  79. When is ACD seen and how is it different from iron deficiency?
    ACD - osteomyelitis, malignancy

    It's NOT iron deficiency because the TIBC is low saying "we have iron but we can't use it"
  80. In Iron deficiency anemia, is the TIBC high or low?
    TIBC is high - it's ready for iron!
  81. Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Iron Deficiency
    • SI: low
    • TIBC: high
    • Ferritin: low
    • % Sat: Super Low
  82. Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: ACD
    • SI: low
    • TIBC: low (can't use it)
    • Ferritin: high (we've got it stored)
    • % Sat: -
  83. Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Hemochromatosis
    • SI: high
    • TIBC: low
    • Ferritin: high
    • % sat: high

    Shitloads of iron!!
  84. Serum Iron, Transferrin or TIBC, Ferritin and % saturation in: Lead Poisoning
    • SI: high
    • TIBC: low
    • Ferritin: -
    • % sat: high
  85. What should you rule out in a male over 50 with new onset iron deficiency anemia?
    colon cancer
  86. What test can be used to diagnose B-thalassemia minor?
    HbA2 will be elevated
  87. Folate Deficiency: Disorder and Findings
    Megaloblastic Anemia

    alcoholism, malnutrition, drugs (methotrexate), pregnancy

    Hypersegmented neutrophils! Glossitis, increased homocysteine
  88. B12 deficiency: Disorder and Findings
    Megaloblastic Anemia

    Vegans, pernicious anemia, fish tapeworm

    Hypersegmented neutrophils - neurological symptoms! increased homocysteine
  89. If a patient has megaloblastic anemia but they are not folate or B12 deficient, what is the disease?
    Orotic Aciduria
  90. What are some causes of nonmegaloblastic macrocyctic anemias?
    • Liver disease - alcoholism
    • orotic aciduria
    • congenital deficiencies of purine/pyrimadine synthesis, drugs which increase erythropoietin
  91. Anemia of Chronic Disease (ACD): Disorder and Findings
    Increased inflammation causes increased hepcidin from liver which prevents release of iron from macrophages

    can go to microcytic hypochromic
  92. Aplastic Anemia: Disorder and Findings
    Nonhemolytic normocytic anemia

    • Due to destruction of myeloid stem cells:
    • radiation, drugs, viral agents, Fanconi's anemia (DNA repair defect)

    Bone marrow gives up - see lots of fat, few cells.

    Sx: fatigue, malaise, bleeding, petechiae, purpura, infection

    Tx: marrow transplant
  93. What viruses can cause aplastic anemia?
    • Parvo B19
    • EBV
    • HIV
    • HCV
  94. Intravascular Hemolysis: Pathogenesis and Findings
    decreased haptoglobin, increased LDH and hemoglobin in urine

    Paroxysmal nocturnal hemoglobinuria, mechanical destruction due to prosthetic valve
  95. Extravascular Hemolysis: Pathogenesis and Findings
    Macrophages in the spleen clear the RBCs - increased LDH and unconjugated bilirubin -> jaundice. Hereditary spherocytosis, G6PD deficiency, sickle cell
  96. Hereditary spherocytosis: Description, Findings and Tx:
    Intrinsic Hemolytic Normocytic Anemia

    defect in ankyrin and spectrin

    anemia, jaundice, pigmented gallstones, splenomegaly, aplastic crisis (Parvo B19)

    Howell Jolly bodies and increased osmotic fragility test

    Tx: Splenectomy
  97. Coombs Tests: Direct vs. Indirect
    Direct - antibody finds antibody - used in hemolytic anemias

    Indirect - patient serum finds antibodies to foreign blood
  98. Cold Agglutinins vs. Warm Agglutinins
    • Cold: IgM - MMM Cold Ice Cream
    • low temp agglutination
    • Mycoplasma Pneumonia, EBV, Malignancy, CLL

    • Warm: IgG Warm is GGGreat!
    • Viruses (EBV, HIV)
    • SLE
    • Malignancies (CLL), nonHodgkin Lymphoma
    • Congential Abnormalities
  99. G6PD Deficiency: Description/Defect, Findings and Tx:
    Intrinsic Hemolytic Normocytic Anemia

    x linked

    • RBC increased oxidative stress
    • sulfa drugs, infections and fava beans
    • Heinz Bodies and Bite Cells

    Back Pain then Peeing Blood
  100. Pyruvate kinase deficiency: Description/Defect, Findings and Tx:
    Intrinsic Hemolytic Normocytic Anemia

    Decreased ATP

    Hemolytic anemia of newborn
  101. Paroxysmal Nocturnal Hemoglobinuria: Defect, Findings and Tx
    Intrinsic Hemolytic Normocytic Anemia

    complement mediated RBC lysis

    CD55 and CD59 missing, Hams test

    Labs show increased hemosiderin in urine
  102. Sickle Cell Anemia: Defect, Findings, Tx
    Intrinsic Hemolytic Normocytic Anemia

    HbS point mutation, low O2 or dehydration causes sickling

    Crew cut on skull (increased erythropoiesis), aplastic crisis, autosplenectomy (increased risk of infection with encapsulated organisms), Howell-Jolly bodies, Salmonella osteomyelitis, renal papillary necrosis

    Tx: hydroxyurea, bone marrow transplant
  103. Autoimmune Hemolytic Anemia: Describe
    Extrinsic Hemolytic Normocytic Anemia

    Many different disease: Erythroblastosis Fetalis, EBV, SLE, CLL, drugs...

    Whenever the immune system decides to attack RBC's - autoimmune hemolytic anemia
  104. Microangiopathic Anemia: Defect, Findings
    RBCs damaged when going through small vessel.

    DIC, TTP/HUS, SLE, Malignant HTN.

    Schistocytes
  105. Macroangiopathic Anemia: Defect and findings
    Prosthetic Heart Valves

    Big Vessels

    Schistocytes
  106. What are two protozoal diseases that can cause hemolytic anemia?
    • Malaria
    • Babesiosis
  107. In what hematologic disorder would you find the following abnormal tests?
    Ham's Test
    DEB test
    Heinz Bodies
    Basophilic Stippling
    Osmotic Fragility Test
    • Hams - PNH
    • DEB - Fanconi's Anemia
    • Heinz - G6PD
    • Basophilic stippling - Lead Poisoning, Iron Deficieincy, ACD, Thalassemia
    • Osmotic Fragility Test - Hereditary Spherocytosis
  108. Platelets: Derived from _, life span of _
    • Megakaryocytes
    • 8-10
  109. What is the vWF receptor on a platelet? What is the fibrinogen receptor?
    • vWF - GpIb
    • Fib - GpIIb/IIIa
  110. Name 4 Platelet Disorders
    • Bernard Soulier Disease
    • Glanzmann's Thrombasthenia
    • Idiopathic Thrombocytopenic Purpura
    • Thrombotic Thrombocytopenic Purpura
  111. Bernard-Soulier Disease: PC, BT, Defect
    ADHESION DEFECT

    Platelet Count decreased, Bleeding Time increased

    defect in Gp1b (can't bind vWF)
  112. Glanzmann's thrombasthenia: BT, Defect
    AGGREGATION defect

    BT increased

    Defect in GpIIb/IIIa

    *No Thrombocytopenia
  113. Idiopathic Thrombocytopenic Purpura: PC, BT, Defect
    Decreased PC, Increased BT

    Autoantibodies to GpIIb/IIIa - platelet destruction

    See Increased Megakaryocytes

    Tx: Steroids or IV IgG
  114. Thrombotic Thrombocytopenic Purpura: PC, BT, Defect, Labs, Symptoms
    AGGREGATION

    PC decreased, BT increased

    Defect ADAMTS 13 - can't degrade vWF

    Labs: schistocytes, increased LDH

    • Symptoms: Pentad
    • 1) Neuro
    • 2) Renal
    • 3) Fever
    • 4) Thrombocytopenia
    • 5) MIHA (microangiopathic hemolytic anemia)

    Very closely related to Hemolytic Uremic Syndrome (HUS)
  115. Hemolytic Uremic Syndrome: Symptoms and bug association
    • Assoc: E coli 0157:H7
    • Sx:

    • 1) Kidney Involvement!
    • 2) Neuro
    • 3) Fever
    • 4) Hemolytic Anemia
    • 5) Thrombocytopenia
  116. von Willebrand's Disease: MOA and Tx
    ADHESION defect

    MOA: Intrinsic Pathway coagulation defect, autosomal dominant

    Tx: DDAVP releases vWF stored in epthelial cells
  117. DIC: Tests, MOA, Causes, Labs
    Tests: Decreased PC, increased BT, PT and PTT

    MOA: widespread clotting activation -> depletes clotting factors -> bleed out

    • Causes: STOP Making New Thrombin:
    • S - Sepsis
    • T - Trauma
    • O - Obstetric Complications
    • P - Pancreatitis
    • M - Malignancy
    • N - Nephrotic Syndrome
    • T - Transfusion

    Labs: Schistocyes, D dimers, decreased fibrinogen, factors V and VIII.
  118. What pathway does PTT correspond to? PT?
    • PT - Extrinsic. "The EX PresidenT went to WARfarin"
    • PTT - Intrinsic
  119. What is PTT? What is PT?
    PTT - Partial Thromboplastin Time.

    PT - Prothrombin Time
  120. NSAIDS inhibit what substance important in platelet aggregation?
    Thromboxane A2
  121. Aspirin: MOA, Clin Use, Tox
    MOA: irreversible COX inhibition

    Clin Use: anti-platelet

    Tox: Gastric Bleeding, Reye's Syndrome, Tinnitus
  122. Clopidogrel and Ticlopidine: MOA, Clin Use, Tox
    MOA: irreversible ADP receptor inhibition

    Clin Use: ACS, coronary stenting,

    Tox: neutropenia (ticlopidine)
  123. Cilostazol, dypyridamole: MOA, Clin Use, Tox
    MOA: Phosphodiesterase III inhibitor

    Clin Use: claudication, stroke prevention, angina prophylaxis

    Tox: flushing, abd pain
  124. Abciximab: MOA, Clin Use, Tox
    MOA: MAB binds GP IIb/IIIa - prevents aggregation

    Clin Use: ACS

    Tox: Thrombocytopenia
  125. Tirobifan and Eptifibatide: MOA
    binds GpIIb/IIIa
  126. Leukemia vs. Lymphoma
    Leukemia - bone marrow involvement. Tumor cells in blood

    Lymphoma - tumor from lymph nodes
  127. Leukemoid Reaction: define
    increased WBC and leukocyte alk phos due to infection, not neoplasia
  128. Hodgkin vs. Non Hodgkin Lymphoma
    • Hodgkin:
    • Reed-Sternberg Cells
    • Local Single Group of Nodes
    • Low Grade Fever, Night Sweats, Weight Loss
    • Non-Tender Mediastinal Lymphadenopathy
    • 50% EBV associated
    • Bimodal Distribution Young or Old

    • Non Hodgkin:
    • HIV and Immunosuppression
    • B Cell Lymphomas
    • Middle Age
  129. What is the most common lymphoma in US adults?
    Diffuse Large B cell lymphoma
  130. What lymphoma is associated with Reed Sternberg Cells?
    Hodgkin Lymphoma
  131. What two lymphomas are associated with EBV?
    Burkitt and Hodgkin
  132. What is the lymphoma equivalent of CLL?
    small lymphocytic lymphoma
  133. What disease has the "starry-sky" pattern due to phagocytosis of apoptotic tumor cells?
    Burkitt Lymphoma
  134. What hematological disease is associated with Sjogren syndrome, Hashimotos thyroiditis and H. pylori?
    Marginal cell MALToma
  135. What is the most common type of Hodgkin Lymphoma?

    Describe RS ratio, prognosis.
    Nodular Sclerosing, Low RS ratio

    Excellent prognosis
  136. Burkitt's Lymphoma: Age group, Genetics, Path
    Non-Hodgkin Lymphoma

    young adults

    t (8:14), c-myc

    Starry Sky Appearance, Associated with EBV, Jaw Lesion, co infection with HIV
  137. Diffuse Large B Cell Lymphoma
    Non Hodgkin Lymphoma

    older adults

    Most common adult NHL.
  138. Mantle Cell Lymphoma: Age, Genetics, Prognosis
    Non Hodgkin Lymphoma

    Older men

    t (11:14), Deactivation of cyclin D

    Poor prognosis
  139. Follicular Lymphoma: Age, Genetics
    NHL

    Adults

    t(14;18), bcl-2 expression (inhibits apoptosis)
  140. Adult T Cell Lymphoma: Age, Genetics, Presentation
    NHL

    Adults

    Caused by Viral infection HTLV-1

    cutaneous lesion presentation. Aggressive
  141. Acute Lymphoblastic Leukemia: Age, Details
    "Blasts" = Acute

    • Children
    • Bone Pain
    • Good Prognosis
    • PAS+
    • Associated with Down's Syndrome (We ALL go DOWN together)
  142. Acute Myeloblastic Leukemia: Age, Details
    "Blasts" = Acute

    • Older Adults 60yo+
    • Auer Rods
    • PAS -
    • t(15;17) -> M3 variant responds to retinoic acid
  143. AML Risk Factors:
    • Radiation, Benzene, Alkylating Agents
    • Aplastic Anemia
    • Down, Fanconi or Bloom Syndrome

  144. Identify cell and associated disease
    Reed Sternberg (Owl Eye)

    Hodgkin Lymphoma

  145. Identify cell and associated disease
    "smudge cells"

    CLL

  146. Identify cell and associated disease
    hairy cell leukemia

    "hair cells"

  147. Identify cell and associated disease
    Auer rods seen in myeloblasts

    AML
  148. Chronic Lymphocytic Leukemia: Age, Cell types and markers
    • Age: over 50
    • B cell markers
    • Smudge cells
    • Warm and cold agglutinins
  149. Chronic Myelogenous Leukemia: Age, Genetics, Symptoms and Treatment
    Age: 30-60y/o

    May progress to AML in 80% of cases

    Philadelphia Chromosome is ALWAYS present (Philadelphia CreaML Cheese)

    Fatigue, splenomegaly, "blast crisis" -> AML,

    Tx: Imatinib
  150. Name the 4 chronic myeloproliferative disorders
    Polycythemia vera - abnormal clone of hematopoeitic stem cells

    Essential Thrombocytosis - megakaryocytes go out of control

    Myelofibrosis - fibrotic obliteration of bone marrow (Teardrop cell)

    CML - Philadelphia Chromosome
  151. Langerhans Cell Histiocytosis: Path and Genetics
    Proliferative disorder of dendritic cells

    Birbeck Granules "tennis rackets" on EM

    Cells express S100 (skin and neuro) and CD1a
  152. t(9;22) associated with:
    CML - Philadelphia Chromosome

    'Tx: Imatinib
  153. t(8;14) associated with:
    Burkitt's Lymphoma (c-myc)
  154. t(14;18) associated with:
    Follicular Lymphomas (bcl-2 activation)
  155. t(15;17) associated with:
    M3 AML - auer rods, DIC, trans retinoic acid
  156. t(11;22) associated with:
    Ewing's Sarcoma
  157. t(11;14) associated with:
    Mantle Cell Lymphoma
  158. t(8;21) associated with:
    AML - Down Syndrome (extra chance to mutate due to trisomy 21)
  159. Multiple Myeloma: Associations, Genetics, Age
    Most common tumor of bone in elderly

    • Lytic lesions
    • Bence Jones proteins in urine (not on basic UA)
    • Rouleaux Formation (Stacks of RBCs)
    • "Clockface Chromatin"
    • Monoclonal plasma cell "fried egg" appearance - shitloads of one type of antibody

    CRAB Symptoms: Hypercalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back Pain
  160. MGUS:
    monoclonal plasma cell expansion without any symptoms
  161. Most common leukemia in children
    ALL
  162. Most common leukemia in adults
    CLL
  163. AML associated with Down Syndrome
    t8;21, AML M7
  164. AML that are CD13 and CD33+
    M0-M6
  165. AML with Characteristic Auer Rods
    M3
  166. Myelodysplastic syndromes have a tendency to progress to:
    AML - blast crisis
  167. Myeloproliferative disorders may progress to
    AML
  168. Greater than 20% blasts suggest:
    Acute Leukemias ALL and AML
  169. Leukemia with more mature cells and less than 5% blasts
    Chronic Leukemias
  170. AML that is CD41 and CD61 +
    AML M7
  171. PAS + acute leukemia
    ALL
  172. Leukemia that commonly presents with bone pain
    ALL
  173. Lekemia equivalent of Burkitt Lymphoma
    ALL L3
  174. Numerous basophils, splenomegaly and negative for leukocyte alkaline phosphatse
    CML
  175. Most common neonatal leukemia
    AML M7
  176. Always positive for philadelphia chromosome
    CML
  177. Only AML that is CD13 and CD 33-
    AML M7
  178. Acute leukemia positive for peroxidase
    AML
  179. Solid sheets of lymphoblasts in marrow
    ALL
  180. PAS (-) acute leukemia
    AML
  181. Always associated with BCR-ABL genes
    CML

Card Set Information

Author:
 cherubicsiren
ID:
 154755
Filename:
 HEME
Updated:
2012-05-19 22:41:08
Tags:
HEME
Folders:

Description:
HEME
Show Answers:

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