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Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. Which infections can cause MAHA?
Extravascular
Signs and sx of aplastic anemia
Valine for glutamate
Malaria - Babesia

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

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3. What is the characteristic lab finding on electrophoresis
Anti Rh
VWF carries/protects factor VIII
Paraprotein spike - monoclonal protein
Gp1b

4. What is the effected enzyme in acute intermittment porphyria?
1 to 6
B12/folate def
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Porphobilinogen deaminase aka uroporphyrinogen I synthase

5. When is the peak incidence for nonHod lymphoma
Nodular sclerosing
Mycosis fundgoides/Sezary syndrome
Easy gas exchange
20 to 40

6. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
CML to AML or All
CD15 and CD30 pos
No lytic bone lesions in WM
Alpha thal - asian and african american

7. Who are the professional APCs?
Inc HbF and dec HbS
B cells
Dendritic cells?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

8. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Porphobilinogen - delta ALA - uroporphyrin
Howell Jolly bodies
Beta thal
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

9. Sickle cell
Intravascular
Sickle cell anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Epoxide reductase - warfarin inhibits

10. Where to T cells arise and mature - and migrate to...
Degrades fibrin mesh and converts C3 to C3a
Schistocytes and inc LDH
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Round densly staining nucleus with a small amount of pale cytoplasm

11. What does Vit K deficiency cause?
Yes - via MHC II
Dec synthesis of factors 1972 - protein C/S
Bernard soulier
Varying shapes

12. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
MGUS - monoclonal gammopathy of undetermined significance
ATIII def
Multiple myeloma

13. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Low in CML
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Blood

14. What is a blast crisis
CML to AML or All
Hereditary spherocytosis - autoimmune hemolysis
Down - down - up
B6 therapy (pyrodixine)

15. What are the neuro sx of B12 def?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Varying sizes
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Schistocytes and inc LDH

16. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Her next Rh+ fetus
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Multiple myeloma
Fibrinogen

17. discrete tumor masses arising from lymph nodes
Pos
Fibrinogen bind GpIIb/IIIa and links platelts
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Lymphoma

18. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Easy gas exchange
Hodgkin
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Contiguous

19. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Langerhans cell histiocytosis
Inc HbA2 on electrophoresis
Fibrinogen bind GpIIb/IIIa and links platelts

20. What is hemophilia B?
120 days
Protoporphyrin
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Def in factor IX

21. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
DIC
Signs and sx of aplastic anemia
B12/folate def
Porphyrias

22. What does STOP Making New Thrombi stand for
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Varying shapes
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Intravascular

23. What is the most common hodgkin lymphoma
Nodular sclerosing
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
2 to 10
SLE - CLL - alpha methyldopa

24. Eosinophils are highly phagocytic For what kind of complex?
Myelofibrosis (marow is crying being its fibrosed'
VWF carries/protects factor VIII
Antigen - antibody
Inactivates it

25. What signal activates MACS
B12 in fatty acid pathways leads to subacute combined degeneration
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
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 -
IFN gama

26. What is factor V leidin?

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27. What is appropriate absolute polycythemia
Inc RBC - dec O2 sat - inc EPO
Essential thrombocytosis
Sideroblastic anemia
T(9;22) bcr abl

28. What do the platelets bind? What is the step called
They bind vWF via GpIb
Ferrocheletase and ALA dehydrogenase
T(9;22) bcr abl
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

29. What is hemophiliia A
Lacunar in nodular sclerosing variant
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Deficiency in factor VIII
DIC

30. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Plasma cell
Protein C or S deficiency
Howell - Jolly bodies - hypo/asplenia
ATIII def

31. What do the dense granules contain in platelets
Aspirin
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
ADP and Ca
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

32. What does plasmin do?
No antigen - both antibodies
Degrades fibrin mesh and converts C3 to C3a
90% anearobically from glucose to lactate - 10% from HMP shunt
They bind vWF via GpIb

33. What is contained within the azurophilic granules of PMNs
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Glanzmann's throbmasthenia
Her next Rh+ fetus
Tissue factor converst the VII and VIIa

34. What is the pattern of involvement and spread for nonHod lympho
Epoxide reductase - warfarin inhibits
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Schistocytes and inc LDH
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

35. What is relative polycythemia
Dec plasma volume
Protein S - cleaves and invactivates Va and VIIIa
Fibrinogen bind GpIIb/IIIa and links platelts
Leukemoid reaction

36. What is the life span of a normal RBC
Parvovirus
120 days
T(12;21)
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

37. What are some classic examples of extravascular hemolysis
Extrinsic - I - II - V - VII and X
Up - down - up
TRAP (tartrate resistant acid phosphatase
Hereditary spherocytosis - G6PD - sickle cell

38. Deficiency in GpIb
Glucose and heme - which inhibit ALA synthase
Dendritic cells?
Folate def
Bernard soulier

39. What is the treatment for sideroblastic anemia
Hairy cell leukemia
B6 therapy (pyrodixine)
Dendritic cells?
Peroxidase

40. What is appropriate absolyte polycythemia associated with
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Blistering cutaneous photosens - most common porphyria
Lung disease - congenital heart diseaes - and high altitude

41. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
ADP and Ca
TXA2 - dec blood flow - inc platelet aggregation
VWD
Intravascular hemolysis

42. What do labs show in DIC?
Schistocytes - helmet cells
Uroporphyrinogen decarboxylase
Inc large vWF multimers - inc platelet aggregation and thrombosis
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

43. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
Ewing sarcoma
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Hereditary spherocytosis - autoimmune hemolysis

44. What is the receptor for fibrinogen?
TRAP (tartrate resistant acid phosphatase
CNS and testis
Parvovirus
GpIIb/IIIa

45. What converts plasminogen to plasm and What does plasmin do?
Blistering cutaneous photosens - most common porphyria
MGUS - monoclonal gammopathy of undetermined significance
DIC - TTP/HUS - traumatic hemolysis
TPA - cleavage of fibrin mesh

46. Blood type A
A antigen and B antibodies
CD15 and CD30 pos
Heinz bodies - seen in alpha thal and G6PD
Round densly staining nucleus with a small amount of pale cytoplasm

47. What is the treatment for acute intermittent porphyria
Blistering cutaneous photosens - most common porphyria
Glucose and heme - which inhibit ALA synthase
Beta thal
DIC

48. How is beta thal minor dx
Inc HbA2 on electrophoresis
Helminth infections major basic protein
VWF carries/protects factor VIII
Up - down - up

49. What is the philadelphia chromosome
T(9;22) bcr abl
Von Willebrand's disease
Inc large vWF multimers - inc platelet aggregation and thrombosis
Myelofibrosis (marow is crying being its fibrosed'

50. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Peroxidase
Parvovirus
Lung disease - congenital heart diseaes - and high altitude
Polycythemia vera