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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. What condition can result from treating AML M3 from the release of the Auer rods
DIC
Ticlopidine/clopidogrel
Mantle cell lymphoma
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

2. What is the most common nonhodgkin lymphoma
Helminth infections major basic protein
Diffuse Large b cell lymphoma
Down - down - up
Orotic aciduria

3. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Tissue
Polycythemia vera
PGI2 - NO inc blood flow - dec platelet aggregation
Down - down - up

4. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
Histaminase and arylsulfatase
MGUS - monoclonal gammopathy of undetermined significance
Round densly staining nucleus with a small amount of pale cytoplasm

5. What is the aggregation phase of ppf?
Fibrinogen bind GpIIb/IIIa and links platelts
Plummer - vinson syndrome
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Paroxysmal nocturnal hemoglobinuria

6. What finding you do you see in patients after splenectomy
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
MAHA
Howell Jolly bodies
G6PD

7. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Inactivates it
Down - down - up
Round densly staining nucleus with a small amount of pale cytoplasm
Nl PT - elevated PTT - intrinsic pathway defect

8. What are the anti aggregation factors?
Dec
They bind vWF via GpIb
PGI2 - NO inc blood flow - dec platelet aggregation
Blod - tissue - MACS

9. Serum iron - transferrin - ferritin lab values for hemochromatosis
Idiopathic thrombocytopenia (ITP)
Bimodal - men>women - except for nodular sclerosing type
Up - down - up
40 to 75

10. When is the peak incidence for nonHod lymphoma
20 to 40
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Dec synthesis of factors 1972 - protein C/S
Hereditary spherocytosis

11. What is the affected enzyme in lead poisoning
B12 def
Mantle cell - older males
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Ferrocheletase and ALA dehydrogenase

12. What is the accumulated substance in lead poisoning
Protoporphyrin (blood)
Sickle cell
Inappropriate absolute with inc RBCs and EPO
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

13. What is the treatment for lead poising?
B12/folate def
HbSS
Myelofibrosis (marow is crying being its fibrosed'
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

14. From what cells are platelets derived from
Megakaryocytes
VWF and fibrinogen
CNS and testis
Von Willebrand's disease

15. What do the platelets bind? What is the step called
They bind vWF via GpIb
Sideroblastic anemia
Decrease EPO
Inc lymphoctes - and less RS cells

16. What do labs show in ITP?
Inducers of primary antibody response
Dec synthesis of factors 1972 - protein C/S
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Burkitt lymphoma

17. What is factor V leidin?

18. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Inc HbA2 on electrophoresis
Inc serum iron - normal TIBC - inc ferratin
Acts to stabilize platelet plug
Hereditary spherocytosis

19. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
DIC
No lytic bone lesions in WM
VWF and fibrinogen
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

20. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Bimodal - men>women - except for nodular sclerosing type
Mantle cell lymphoma
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Nl - up - nl

21. What is the pathogenesis of sickle cell
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Pyruvate kinase def - extravascular
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Imatinib - anti bcr abl

22. What is the effected enzyme in acute intermittment porphyria?
Pyruvate kinase def - extravascular
Lysine for glutamate at position 6
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 -
Porphobilinogen deaminase aka uroporphyrinogen I synthase

23. t(11:22)
Essential thrombocytosis
5- FU - AZT - hydroxyurea
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Ewing sarcoma

24. What do the iron studies show in sideroblastic anemia
MHC II and Fc receptors
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Glucose and heme - which inhibit ALA synthase
Inc serum iron - normal TIBC - inc ferratin

25. Upregulated growth of leukocytes in bone marro
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Leukemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Antigen - antibody

26. discrete tumor masses arising from lymph nodes
Petechiae
Lymphoma
Leukemoid reaction
MAHA

27. What is the are the presenting symptoms of lead poisoning in kids and adults
Inc
Leukemoid reaction
Schistocytes and inc LDH
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

28. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Nl PT - elevated PTT - intrinsic pathway defect
Fc
DIC
HbSS

29. what proteins can be defective in HS?
Ankryin - band 4.1 or spectrin
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Beta chain underproduced - asymptomatic - heterozygote
G6PD

30. What is relative polycythemia
Paroxysmal nocturnal hemoglobinuria
Exposed collagen upon endothelial damage
Dec plasma volume
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

31. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
Heinz bodies - seen in alpha thal and G6PD
Bimodal - men>women - except for nodular sclerosing type
Bernard soulier

32. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Extravascular
Varying sizes
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Histamine - heparin - and eosinohil chemotactic factors

33. What is the philadelphia chromosome
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 -
T(9;22) bcr abl
B6
Splicing sites and promotor sequences

34. What the alpha granules contain in platelets?
Howell Jolly bodies
VWF and fibrinogen
Hodgkin
Gp1b

35. Basophilica stippling
T(12;21)
Protein S - cleaves and invactivates Va and VIIIa
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Inc RBC - dec O2 sat - inc EPO

36. HTLV-1
T(12;21)
Adult T cell lymphoma - presents with cutaneous lesions
Factor V Leiden
Abciximab

37. What are some classic examples of extravascular hemolysis
EBV
Hereditary spherocytosis - G6PD - sickle cell
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
2 to 10

38. What is the difference of presentation of Burkitt in Africa vs the United States
B6
Bite cells and Heinz bodies
Africa = Jaw lesion - US = pelvis or abdomen
ATIII def

39. How does vWD cause elevated PTT?
HIV or immunosupression
VWF carries/protects factor VIII
B12 in fatty acid pathways leads to subacute combined degeneration
Polycythemia vera

40. Macro - ovalocyte
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Leukemia
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

41. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
G6PD
Cromolyn sodium

42. What virus is associated with 50% of Hodgkin lymphoma
Porphyrias
EBV
All
Inc vasodiltion - inc perm - inc pain

43. Where are monocytes typically found - where do they go - and What do the differentiate into?
Intravascular
Blod - tissue - MACS
Fibrinogen
Indirect coombs - agglutinate if serum anti RBC surface Ig

44. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Intravascular hemolysis
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 -
Nl - up - nl

45. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Inappropriate absolute with inc RBCs and EPO
Paroxysmal nocturnal hemoglobinuria
Helps platelts adhere to endothelium
Dec plasma volume

46. What is a metabolic disorder tht can cause macrocytic anemia?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
...
Ticlopidine/clopidogrel
Orotic aciduria

47. What are the labs and tx for HS?
40 to 50
Positive osmootic fragility test and splenectomy
Inc RBC - dec O2 sat - inc EPO
Bernard soulier

48. What begins the extrinsic pathway?
G6PD
Tissue factor converst the VII and VIIa
Incactivates II - VII - IX - X - XI - XII
B cells

49. What substance prevents mast cells degranulation?
Factor V Leiden
Cromolyn sodium
Produce antibody - lots of RER and golgi
B antigena and A antibodes

50. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
G6PD
GpIIb/IIIa
Paraprotein spike - monoclonal protein