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Hemeonc

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Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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1. tennis rackets on EM
Positive osmootic fragility test and splenectomy
Birbeck granules
Inc UCB
Gp1b

2. What percentage of WBCs are monocytes?
Aspirin
Imatinib - anti bcr abl
2 to 10
Low O2 in papilla; can also get microhematuria from medullary infarcts

3. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
...
Beta thal
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

4. Where are mast cells found
Tissue
Down - up - down
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Ewing sarcoma

5. Plasma cell neoplasm
Multiple myeloma
Degrades fibrin mesh and converts C3 to C3a
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Cromolyn sodium

6. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
No platelet clumping
Heinz bodies - seen in alpha thal and G6PD
Nl - up - nl

7. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Inc HbA2 on electrophoresis
Alpha thal - asian and african american
DIC
Acts to stabilize platelet plug

8. What is the ddx for aplastic anemia

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9. What virus can cause an aplastic crisis in pts with HS?
VIII
Protein C or S deficiency
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Parvovirus

10. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Deficiency in factor VIII
1 to 6
Extravascular hemolysis
Down - down - up

11. How does the therapy for M3 vairant work?
Thrombotic thrombocytopenic purpura
Induces differentiation of myeloblasts
Intravascular hemolysis
No lytic bone lesions in WM

12. Basophilica stippling
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Paroxysmal nocturnal hemoglobinuria
Gp1b

13. Fibrotic obliteration of bone marow with teardrop cells

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14. Is HS extravascular or intravascular?
Bleeding - malnutrition/absorption - inc demand pregs
Extravascular
Easy gas exchange
Polycythemia vera

15. What is the result of thrombocytopenia or platelet dysfunction?
Varying shapes
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
1 to 6
Petechiae

16. Wilm's tumor - RCC - HCC - hydronephrosis
Down - up - down
Aplastic anemia - pancytopenia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Glanzmann's throbmasthenia

17. Who are the professional APCs?
Dendritic cells?
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Deficiency in factor VIII
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

18. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
No lytic bone lesions in WM
Examples of oxidative stress

19. What is makes a leukemia acute
HbSS
Heinz bodies - seen in alpha thal and G6PD
Blasts > 5%
Bimodal - men>women - except for nodular sclerosing type

20. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Ringed sideronblasts with iron laden mitochondria
Anti Rh
120 days
Beta chain underproduced - asymptomatic - heterozygote

21. Conditions of defective heme synthesis leading to accumulation of heme precurors
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Porphyrias
Protein C or S deficiency
Beta chain underproduced - asymptomatic - heterozygote

22. When do you see MAHA?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
MGUS - monoclonal gammopathy of undetermined significance
Inactivates it
B antigena and A antibodes

23. What activates the intrinsic pathway?
Pyruvate kinase def - extravascular
Tissue factor converst the VII and VIIa
Howell Jolly bodies
Collagen - BM - activated platelets

24. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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25. Which factor does vWF carry/protect
Intravascular
Inc HbF and dec HbS
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
VIII

26. What is hemophiliia A
Deficiency in factor VIII
Protoporphyrin
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Multiple myeloma

27. Upregulated growth of leukocytes in bone marro
Macrohemorrhage - hemarthroses - easy bruising
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Intravascular
Leukemia

28. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Paroxysmal nocturnal hemoglobinuria
Thrombotic thrombocytopenic purpura
Hydroxyurea - bone marrow transplant
Von Willebrand's disease

29. What causes renal papillary necrosis in sickle cell
Nl - up - nl
Low O2 in papilla; can also get microhematuria from medullary infarcts
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
No

30. Drug that inhbits the GpIIb/IIIa directly
Parvovirus
Blistering cutaneous photosens - most common porphyria
Abciximab
GpIIb/IIIa

31. What is the clinical picture of hemophilia A or B
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
AML
Macrohemorrhage - hemarthroses - easy bruising
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

32. What is a blast crisis
CML to AML or All
Parvovirus
Helminth infections major basic protein
B6

33. Sickle cell
Lung disease - congenital heart diseaes - and high altitude
Protein S - cleaves and invactivates Va and VIIIa
Sickle cell anemia
90% anearobically from glucose to lactate - 10% from HMP shunt

34. Ringed sideroblasts
40 to 50
Ringed sideronblasts with iron laden mitochondria
Sideroblastic anemia
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

35. What does CRAB stand for in multiple myeloma
Diffuse Large b cell lymphoma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
All

36. What are the main associations with multiple myeloma?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Imatinib - anti bcr abl
Lead - and EtOH
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

37. What is the activation stage of platelet plug formation?
G6PD
Glanzmann's throbmasthenia
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Antigen - antibody

38. Where does All spread
CNS and testis
They bind vWF via GpIb
Lysine for glutamate at position 6
Indirect coombs - agglutinate if serum anti RBC surface Ig

39. What portion of IgE can mast cells bind
Kids= exposure to lead paint - adults = battery - ammunition factory
Folate def
Fc
Protein C or S deficiency

40. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Anemia of chronic disease - Aplastic anemia - kidney disease
Blood
Liver disease - abetalipoproteinemia - acntho = spiny
Sideroblastic anemia

41. What is the characteristic spread of Hodgkin Lymphoma
Parvovirus
Imatinib - anti bcr abl
Contiguous
T(12;21)

42. What does LEAD stand for in lead poisoning?
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
B antigena and A antibodes
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Easy gas exchange

43. Megakaryocytosis
Multiple myeloma
Birbeck granules
Intravascular
Essential thrombocytosis

44. Basophilic nuclear remnants fonud in RBCs
Uroporphyrinogen decarboxylase
Howell - Jolly bodies - hypo/asplenia
Leukemia
Low in CML

45. What causes the jaundice in extravascular hemolysis
Birbeck granules
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Inc UCB
Folate def

46. What is factor V leidin?

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47. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Leukemia
Uroporphyrin (tea colored urine)
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

48. What does 'Neutrophils Like Making Everything Better' stand for?
MHC II and Fc receptors
Extrinsic - I - II - V - VII and X
Polycythemia vera
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

49. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Both alpha and beta thal
Inc RBC - dec O2 sat - inc EPO
Low in CML
Inc serum iron - normal TIBC - inc ferratin

50. From what cells are platelets derived from
Factor V Leiden
Megakaryocytes
VWF and fibrinogen
Porphyrias