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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. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
Diffuse Large b cell lymphoma
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Leukemoid reaction

2. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
DIC
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Hereditary spherocytosis - autoimmune hemolysis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

3. What is the pathogenesis of TTP?
A antigen and B antibodies
Nl PT - elevated PTT - intrinsic pathway defect
Porphobilinogen - delta ALA - uroporphyrin
Inc large vWF multimers - inc platelet aggregation and thrombosis

4. What causes renal papillary necrosis in sickle cell
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Low O2 in papilla; can also get microhematuria from medullary infarcts
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 -
Intravascular hemolysis

5. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Dec plasma volume
Splicing sites and promotor sequences
Leukemia
Exposed collagen upon endothelial damage

6. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
B12 def
Round densly staining nucleus with a small amount of pale cytoplasm
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

7. What are the anti aggregation factors?
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
DIC
Nl PT - elevated PTT - intrinsic pathway defect
PGI2 - NO inc blood flow - dec platelet aggregation

8. What does the blood smear show in glanzmann's?
Hereditary elloptocytosis
No platelet clumping
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
AML

9. What is hemophiliia A
Uroporphyrinogen decarboxylase
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
ADP and Ca
Deficiency in factor VIII

10. What activates the intrinsic pathway?
Imatinib - anti bcr abl
Collagen - BM - activated platelets
Peroxidase
Thrombotic thrombocytopenic purpura

11. How does the therapy for M3 vairant work?
Antigen - antibody
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 -
Incactivates II - VII - IX - X - XI - XII
Induces differentiation of myeloblasts

12. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Reed - sternberg cells
Tissue factor converst the VII and VIIa
Epoxide reductase - warfarin inhibits
PGI2 - NO inc blood flow - dec platelet aggregation

13. What is the characteristic spread of Hodgkin Lymphoma
Fe def - thal - pb poison - sideroblastic anemia
Beta chain underproduced - asymptomatic - heterozygote
Contiguous
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

14. What is the ddx for aplastic anemia

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15. Schisotcyte - helmet cell
Burkitt lymphoma
DIC - TTP/HUS - traumatic hemolysis
Common a few days after oxidative stress in a pt with G6PD
Low in CML

16. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
Nl - up - nl
EBV
Hereditary elloptocytosis

17. What are the azuraphilic granules in PMNs
Off center nuclues - clock face chromatin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Lysosomes
Bernard soulier

18. poikilocytosis
Varying shapes
Howell - Jolly bodies - hypo/asplenia
Beta 4
Positive osmootic fragility test and splenectomy

19. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Histaminase and arylsulfatase
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Howell Jolly bodies
Hereditary spherocytosis

20. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Hodgkin
Africa = Jaw lesion - US = pelvis or abdomen
Off center nuclues - clock face chromatin

21. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
VWF and fibrinogen
Up - down - nl
B12/folate def
Blod - tissue - MACS

22. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Blasts > 5%
They bind vWF via GpIb
Dec plasma volume

23. What converts plasminogen to plasm and What does plasmin do?
Sideroblastic anemia
Inactivates it
Uroporphyrin (tea colored urine)
TPA - cleavage of fibrin mesh

24. Is HS extravascular or intravascular?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Extravascular
Howell - Jolly bodies - hypo/asplenia
Fe def - thal - pb poison - sideroblastic anemia

25. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Idiopathic thrombocytopenia (ITP)
Glycine and succinyl - coa
Alpha thal - asian and african american
Polycythemia vera

26. What cell is primarily involved in non Hod lymph
CML
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 cells
T(9;22) bcr abl

27. What do platelets release necessary for coagulation cascaed?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
ADP and Ca
Leukemoid reaction
Down - up - down

28. Where are monocytes typically found - where do they go - and What do the differentiate into?
All
Blod - tissue - MACS
B6 therapy (pyrodixine)
Orotic aciduria

29. What finding you do you see in patients after splenectomy
Antigen - antibody
Off center nuclues - clock face chromatin
Uroporphyrin (tea colored urine)
Howell Jolly bodies

30. What is the enzyme defect in Porphyria cutanea tarda
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Bimodal - men>women - except for nodular sclerosing type
Uroporphyrinogen decarboxylase
ATIII def

31. Adults present with cutaneous patches/nodules - indolent CD4+
Glucose and heme - which inhibit ALA synthase
HbSS
Hereditary spherocytosis - G6PD - sickle cell
Mycosis fundgoides/Sezary syndrome

32. Basophilic nuclear remnants fonud in RBCs
Yes - via MHC II
Inc RBC - dec O2 sat - inc EPO
Howell - Jolly bodies - hypo/asplenia
Anemia of chronic disease - Aplastic anemia - kidney disease

33. What is the mutation in HbS
Valine for glutamate
Helps platelts adhere to endothelium
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

34. What virus is associated with Burkitt lymphoma
EBV
Fibrinogen bind GpIIb/IIIa and links platelts
Down - down - up
Hereditary elloptocytosis

35. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
CD15 and CD30 pos
Essential thrombocytosis
Bimodal - men>women - except for nodular sclerosing type
Down - down - up

36. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
IgG - Warm is GREAT
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Common a few days after oxidative stress in a pt with G6PD
No lytic bone lesions in WM

37. What do the iron studies show in sideroblastic anemia
B12 def
Inc serum iron - normal TIBC - inc ferratin
Round densly staining nucleus with a small amount of pale cytoplasm
Both alpha and beta thal

38. What are the age ranges for the various leukemias
Varying sizes
Inactivates it
Varying shapes
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

39. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Adult T cell lymphoma - presents with cutaneous lesions
All trans retinoic acid

40. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
B12 def
B cells
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

41. Sickle cell
Sickle cell anemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Down - up - down
CD15 and CD30 pos

42. What is the characteristic lab finding on electrophoresis
Africa = Jaw lesion - US = pelvis or abdomen
40 to 50
CNS and testis
Paraprotein spike - monoclonal protein

43. What is the effected enzyme in acute intermittment porphyria?
All
VWF and fibrinogen
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Porphobilinogen deaminase aka uroporphyrinogen I synthase

44. drug that inhibits ADP induced expression of GpIIb/IIIa
Leukemia
Ticlopidine/clopidogrel
Low O2 in papilla; can also get microhematuria from medullary infarcts
All

45. What is the affected enzyme in acute intermittment porphyria
Porphobilinogen - delta ALA - uroporphyrin
SLE - CLL - alpha methyldopa
Hereditary elloptocytosis
HbSS

46. In hemophilia A or B What do you see in the coag tests
Contiguous
Nl PT - elevated PTT - intrinsic pathway defect
Hereditary spherocytosis - G6PD - sickle cell
IFN gama

47. What is their role?
Inducers of primary antibody response
TPA - cleavage of fibrin mesh
Common a few days after oxidative stress in a pt with G6PD
...

48. What does NAACP stand for - in regards to the causes of eosinophiia?
Pyruvate kinase def - extravascular
Liver disease - abetalipoproteinemia - acntho = spiny
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Abciximab

49. What percentage if WBCs are polys?
Hereditary spherocytosis - autoimmune hemolysis
Blistering cutaneous photosens - most common porphyria
CD15 and CD30 pos
40 to 75

50. Which infections can cause MAHA?
Hydroxyurea - bone marrow transplant
Malaria - Babesia
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Porphyrias