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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 chromosomal translocation is associated with a better prognosis in All
T(12;21)
Protoporphyrin (blood)
Exposed collagen upon endothelial damage
Bleeding - malnutrition/absorption - inc demand pregs

2. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Protoporphyrin
B6 therapy (pyrodixine)
No platelet clumping

3. hemolytic in a newborn - dec ATP and rigid RBCs
Pyruvate kinase def - extravascular
B antigena and A antibodes
Anti Rh
Rhogam - Rh antigen immunoglobulin

4. What role does antithrombin play?
Dec plasma volume
Alpha thal - asian and african american
Incactivates II - VII - IX - X - XI - XII
VWD

5. What is the tx for vWD
Diffuse Large b cell lymphoma
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Tissue factor converst the VII and VIIa
Pos

6. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Parvovirus
G6PD
No lytic bone lesions in WM
Induces differentiation of myeloblasts

7. What is the pathogenesis of ACD?
Tissue
Fe def - thal - pb poison - sideroblastic anemia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

8. what proteins can be defective in HS?
Idiopathic thrombocytopenia (ITP)
Valine for glutamate
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Ankryin - band 4.1 or spectrin

9. What do auer rods stain with
Peroxidase
Sideroblastic anemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
CML

10. Basophilic nuclear remnants fonud in RBCs
Helminth infections major basic protein
Inactivates it
Howell - Jolly bodies - hypo/asplenia
20 to 40

11. Drug that inhibits COX and therefore TXA2 synthesis
Aspirin
Protein C or S deficiency
Ringed sideronblasts with iron laden mitochondria
Inc vasodiltion - inc perm - inc pain

12. What percentage if WBCs are polys?
Essential thrombocytosis
40 to 75
Inc lymphoctes - and less RS cells
Diffuse Large b cell lymphoma

13. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Cromolyn sodium
No lytic bone lesions in WM
2 to 10
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

14. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
40 to 75
Imatinib - anti bcr abl
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

15. What are the likely exposures of kids and adults for lead poisoning
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Kids= exposure to lead paint - adults = battery - ammunition factory
B12 in fatty acid pathways leads to subacute combined degeneration

16. What reveresible things can a sideroblastic anemia
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Lead - and EtOH
Glanzmann's throbmasthenia

17. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Ticlopidine/clopidogrel
They bind vWF via GpIb
Sickle cell

18. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Porphyrias
EBV
Induces differentiation of myeloblasts
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

19. What is the treatment to prevent a woman from forming anti Rh antibody?
Glucose and heme - which inhibit ALA synthase
Dec synthesis of factors 1972 - protein C/S
Rhogam - Rh antigen immunoglobulin
Extravascular hemolysis

20. What does the blood smear show in glanzmann's?
MGUS - monoclonal gammopathy of undetermined significance
No platelet clumping
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Blod - tissue - MACS

21. What are the presenting symptoms of acute intermittent porphyria
Inactivates it
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
B6
Off center nuclues - clock face chromatin

22. How does the therapy for M3 vairant work?
G6PD
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Induces differentiation of myeloblasts
Varying sizes

23. What is the affected enzyme in lead poisoning
Factor V Leiden
ADP and Ca
Ferrocheletase and ALA dehydrogenase
Epoxide reductase - warfarin inhibits

24. Elderly - mature b cell tumor with filamentous - hairlike projections
GpIIb/IIIa
Hairy cell leukemia
Complications of sickle cell anemia
Macrohemorrhage - hemarthroses - easy bruising

25. drug that inhibits ADP induced expression of GpIIb/IIIa
Hereditary spherocytosis
Ticlopidine/clopidogrel
Beta 4
Glanzmann's throbmasthenia

26. Where are 1/3 of platelets stored
Sickle cell
Eleveated PT - PTT
Round densly staining nucleus with a small amount of pale cytoplasm
Spleen

27. philadelphia chromosome - blood looks like marrow
Inc HbA2 on electrophoresis
TPA - cleavage of fibrin mesh
G6PD
CML

28. What is the therapy for the M3 variant?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Pyruvate kinase def - extravascular
All trans retinoic acid
40 to 75

29. Where are basophils found?
Blood
Lacunar in nodular sclerosing variant
Varying sizes
Inc

30. What does STOP Making New Thrombi stand for
Plummer - vinson syndrome
Fibrinogen bind GpIIb/IIIa and links platelts
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Protein C or S deficiency

31. What is the activation stage of platelet plug formation?
HIV or immunosupression
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Induces differentiation of myeloblasts
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

32. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Porphobilinogen - delta ALA - uroporphyrin
VWD
Positive osmootic fragility test and splenectomy
DIC

33. What is the characteristic lab finding on electrophoresis
Megakaryocytes
Paraprotein spike - monoclonal protein
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
...

34. Upregulated growth of leukocytes in bone marro
Blod - tissue - MACS
Birbeck granules
Leukemia
Howell - Jolly bodies - hypo/asplenia

35. From what cells are platelets derived from
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Sideroblastic anemia
Glycine and succinyl - coa
Megakaryocytes

36. What cell is primarily involved in non Hod lymph
Hb Barts - gamma4 in defect in all 4 alpha genes
G6PD
B cells
Cromolyn sodium

37. What is the ddx for aplastic anemia

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38. mother's antibodies attack fetal RBCs
CML to AML or All
Schistocytes and inc LDH
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Follicular lymphoma - indolent course

39. What is the pathogenesis of TTP?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Hereditary spherocytosis - G6PD - sickle cell
Inc large vWF multimers - inc platelet aggregation and thrombosis
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

40. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
They bind vWF via GpIb
Folate def
Helps platelts adhere to endothelium
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

41. What do platelets release necessary for coagulation cascaed?
ADP and Ca
Her next Rh+ fetus
Lysosomes
Orotic aciduria

42. t(8;14) c - myc gene
Langerhans cells
Sickle cell
Burkitt lymphoma
Macrohemorrhage - hemarthroses - easy bruising

43. CD5+ - poor prognosis - t(11;14)
Leukemia
Common a few days after oxidative stress in a pt with G6PD
Intravascular hemolysis
Mantle cell - older males

44. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Kids= exposure to lead paint - adults = battery - ammunition factory
Blasts > 5%
Inactivates it

45. universal recipient
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
AB - no antibodies
Up - down - up
Positive osmootic fragility test and splenectomy

46. What is the most common hodgkin lymphoma
Nodular sclerosing
Alpha thal - asian and african american
AML
Epoxide reductase - warfarin inhibits

47. What the alpha granules contain in platelets?
Deficiency in factor VIII
Helminth infections major basic protein
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
VWF and fibrinogen

48. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Langerhans cells
Von Willebrand's disease
Complications of sickle cell anemia
Dec synthesis of factors 1972 - protein C/S

49. What is the treatment for sideroblastic anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
B6 therapy (pyrodixine)
All
Fc

50. Spherocyte
Plummer - vinson syndrome
Hereditary spherocytosis - autoimmune hemolysis
Parvovirus
Common a few days after oxidative stress in a pt with G6PD