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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 is the characteristic finding for MAHA on peripheral smear?
Schistocytes - helmet cells
Dec synthesis of factors 1972 - protein C/S
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Inactivates it

2. What cell is primarily involved in non Hod lymph
Leukemia
B cells
Common a few days after oxidative stress in a pt with G6PD
Sickle cell

3. What is the ddx for aplastic anemia

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4. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Alpha thal - asian and african american
DIC
Langerhans cells

5. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Degrades fibrin mesh and converts C3 to C3a
Spleen
Up - down - up

6. What do you see on peripheral smear with sideroblastic anemia
Langerhans cells
ADP and Ca
Fibrinogen bind GpIIb/IIIa and links platelts
Ringed sideronblasts with iron laden mitochondria

7. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Histamine - heparin - and eosinohil chemotactic factors
Idiopathic thrombocytopenia (ITP)
Antigen - antibody
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

8. What do the dense granules contain in platelets
Fibrinogen
T(12;21)
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
ADP and Ca

9. How does the therapy for M3 vairant work?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
ADP and Ca
Intrinsic - all factors except - VII - XIII
Induces differentiation of myeloblasts

10. Is HS extravascular or intravascular?
Bone marrow infiltration - myelofibrosis
Valine for glutamate
Extravascular
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

11. What are the etiologies of folate def
Inactivates it
Inc large vWF multimers - inc platelet aggregation and thrombosis
Down - down - up
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

12. Is G6PD intravascular or extravascular
AB - no antibodies
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Intravascular
Alpha thal - asian and african american

13. tennis rackets on EM
EBV
Bite cells and Heinz bodies
Birbeck granules
Inc large vWF multimers - inc platelet aggregation and thrombosis

14. What activates the fibrinolytic pathway?
Spleen
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Leukemia
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

15. universal donor
Dendritic cells?
Factor V Leiden
Protein C or S deficiency
No antigen - both antibodies

16. What do they express on their surface?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Hb Barts - gamma4 in defect in all 4 alpha genes
Tissue
MHC II and Fc receptors

17. What virus can cause an aplastic crisis in pts with HS?
Orotic aciduria
Inc HbF and dec HbS
Nodular sclerosing
Parvovirus

18. What does Vit K deficiency cause?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Dec synthesis of factors 1972 - protein C/S
AML
Howell - Jolly bodies - hypo/asplenia

19. What is the effected enzyme in acute intermittment porphyria?
Def in factor IX
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Valine for glutamate
Extravascular

20. What is the Ddx for a normocytic - normochromic anemia?
T(12;21)
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Lacunar in nodular sclerosing variant

21. What are the anti aggregation factors?
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Gp1b
Fe def - thal - pb poison - sideroblastic anemia
PGI2 - NO inc blood flow - dec platelet aggregation

22. What is their role?
Lacunar in nodular sclerosing variant
Uroporphyrinogen decarboxylase
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Inducers of primary antibody response

23. Can B cells function as APCs?
Epoxide reductase - warfarin inhibits
Hb Barts - gamma4 in defect in all 4 alpha genes
Tissue
Yes - via MHC II

24. Causes of iron def?
ATIII def
Bleeding - malnutrition/absorption - inc demand pregs
Adult T cell lymphoma - presents with cutaneous lesions
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

25. What is the coombs test results in AIHA?
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
GpIIb/IIIa
Low in CML
Pos

26. What chromosomal translocation is associated with a better prognosis in All
MGUS - monoclonal gammopathy of undetermined significance
T(12;21)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

27. What role does antithrombin play?
Fc
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Incactivates II - VII - IX - X - XI - XII
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

28. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Tissue factor converst the VII and VIIa
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Spleen

29. What is the life spance of a platelet?
SLE - CLL - alpha methyldopa
CNS and testis
8-10 days
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

30. inc LDH - jaundice
Africa = Jaw lesion - US = pelvis or abdomen
Dec synthesis of factors 1972 - protein C/S
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Extravascular hemolysis

31. What is the pathogenesis of sickle cell
40 to 50
B12/folate def
Indirect coombs - agglutinate if serum anti RBC surface Ig
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

32. Deficiency in GpIIb/IIIa

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33. Blood type A
Eleveated PT - PTT
A antigen and B antibodies
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Round densly staining nucleus with a small amount of pale cytoplasm

34. Which factor does vWF carry/protect
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Common a few days after oxidative stress in a pt with G6PD
VIII
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

35. What are the age ranges for the various leukemias
Nl PT - elevated PTT - intrinsic pathway defect
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Bleeding - malnutrition/absorption - inc demand pregs
Leukemoid reaction

36. What does hairy cell leukemia stain with
CNS and testis
B symptoms - fever night sweats - weight loss
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
TRAP (tartrate resistant acid phosphatase

37. When is the peak incidence for nonHod lymphoma
20 to 40
Complications of sickle cell anemia
Spleen
Fc

38. What activates protein C and What does activated protein C do?
Hereditary spherocytosis
Sickle cell anemia
Mycosis fundgoides/Sezary syndrome
Protein S - cleaves and invactivates Va and VIIIa

39. What do platelets interact with to form a hemostatic plug
Pyruvate kinase def - extravascular
B6 therapy (pyrodixine)
Fibrinogen
Inc serum iron - normal TIBC - inc ferratin

40. anti - Ig antibody added to patients RBCs;
Glycine and succinyl - coa
Sickle cell anemia
Fibrinogen
Direct coombs - agglutinate if RBCs are coated with Ig

41. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
T(9;22) bcr abl
Porphobilinogen - delta ALA - uroporphyrin
IgG - Warm is GREAT

42. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Malaria - Babesia
Epoxide reductase - warfarin inhibits
Bernard soulier
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

43. What is the aggregation phase of ppf?
Intravascular hemolysis
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Anti Rh
Fibrinogen bind GpIIb/IIIa and links platelts

44. 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
Blood
Anemia of chronic disease - Aplastic anemia - kidney disease
Birbeck granules
Sickle cell

45. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
All trans retinoic acid
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Protein C or S deficiency

46. How are plasma cells characterized?
Collagen - BM - activated platelets
Sideroblastic anemia
Off center nuclues - clock face chromatin
Deficiency in factor VIII

47. What is the are the presenting symptoms of lead poisoning in kids and adults
Down - up - down
Megakaryocytes
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Lacunar in nodular sclerosing variant

48. How are the nucleus and the cytoplasm characterized for lymphocytes
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Lacunar in nodular sclerosing variant
Round densly staining nucleus with a small amount of pale cytoplasm
Inc HbA2 on electrophoresis

49. What are the presenting symptoms of porphyria cutanea tarda
Histaminase and arylsulfatase
Common a few days after oxidative stress in a pt with G6PD
Blistering cutaneous photosens - most common porphyria
Anti Rh

50. What is the treatment to prevent a woman from forming anti Rh antibody?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Produce antibody - lots of RER and golgi
Rhogam - Rh antigen immunoglobulin
Inducers of primary antibody response