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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 ddx for aplastic anemia

2. What activates the intrinsic pathway?
Collagen - BM - activated platelets
Down - down - up
Factor V resistant to activated protein C's inhibition
Deficiency in factor VIII

3. What converts plasminogen to plasm and What does plasmin do?
Intravascular hemolysis
TPA - cleavage of fibrin mesh
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Produce antibody - lots of RER and golgi

4. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Tissue factor converst the VII and VIIa
Protoporphyrin
Idiopathic thrombocytopenia (ITP)
Her next Rh+ fetus

5. What does Vit K deficiency cause?
Lead - and EtOH
Aplastic anemia - pancytopenia
Dec synthesis of factors 1972 - protein C/S
Follicular lymphoma - indolent course

6. What is the result of thrombocytopenia or platelet dysfunction?
Anemia of chronic disease - Aplastic anemia - kidney disease
Petechiae
Beta 4
Protein S - cleaves and invactivates Va and VIIIa

7. mother's antibodies attack fetal RBCs
SLE - CLL - alpha methyldopa
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Gp1b
Histamine - heparin - and eosinohil chemotactic factors

8. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Intrinsic - all factors except - VII - XIII

9. What do eosinophils defend against and What do they use to do it?
Glanzmann's throbmasthenia
Hereditary spherocytosis
VWD
Helminth infections major basic protein

10. What is the defect in beta thal?
CML to AML or All
T(12;21)
ATIII def
Splicing sites and promotor sequences

11. What is the purpose of the fibrin mesh
Acts to stabilize platelet plug
Glucose and heme - which inhibit ALA synthase
They bind vWF via GpIb
Inc HbA2 on electrophoresis

12. What is the affected enzyme in lead poisoning
Signs and sx of aplastic anemia
Ferrocheletase and ALA dehydrogenase
Dec
Paraprotein spike - monoclonal protein

13. Where are basophils found?
Up - down - nl
Follicular lymphoma - indolent course
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Blood

14. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
SLE - CLL - alpha methyldopa
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Folate def

15. What is the receptor for vWF
Leukemia
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Gp1b
B12 def

16. What is the activation stage of platelet plug formation?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Anemia of chronic disease - Aplastic anemia - kidney disease
Langerhans cell histiocytosis

17. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Idiopathic thrombocytopenia (ITP)
Inducers of primary antibody response
B6 therapy (pyrodixine)
Intravascular hemolysis

18. Serum iron - transferrin - ferritin lab values for hemochromatosis
Langerhans cells
Plummer - vinson syndrome
Uroporphyrin (tea colored urine)
Up - down - up

19. How does the therapy for M3 vairant work?
Induces differentiation of myeloblasts
Pyruvate kinase def - extravascular
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
20 to 40

20. What percentage of WBCs are eosinophils?
1 to 6
Degrades fibrin mesh and converts C3 to C3a
Africa = Jaw lesion - US = pelvis or abdomen
Howell Jolly bodies

21. What is appropriate absolute polycythemia
Off center nuclues - clock face chromatin
Hereditary spherocytosis
Valine for glutamate
Inc RBC - dec O2 sat - inc EPO

22. What are the likely exposures of kids and adults for lead poisoning
Plummer - vinson syndrome
AB - no antibodies
Kids= exposure to lead paint - adults = battery - ammunition factory
Round densly staining nucleus with a small amount of pale cytoplasm

23. What is the characteristic lab finding on electrophoresis
Essential thrombocytosis
Paraprotein spike - monoclonal protein
Contiguous
Multiple myeloma

24. What is the age group most commonly affected by multiple myeloma
Degrades fibrin mesh and converts C3 to C3a
40 to 50
Dec synthesis of factors 1972 - protein C/S
TXA2 - dec blood flow - inc platelet aggregation

25. What is the pathogenesis of aplastic anemia with kidney disease
Uroporphyrinogen decarboxylase
Decrease EPO
Essential thrombocytosis
Beta 4

26. iron deficiency anemia - esophageal web - atrophic glossitis
Porphyrias
Plummer - vinson syndrome
Lung disease - congenital heart diseaes - and high altitude
Sideroblastic anemia

27. Where are mast cells found
Tissue
TRAP (tartrate resistant acid phosphatase
Langerhans cell histiocytosis
Inappropriate absolute with inc RBCs and EPO

28. What do they express on their surface?
MHC II and Fc receptors
Dec synthesis of factors 1972 - protein C/S
Blod - tissue - MACS
Protoporphyrin (blood)

29. philadelphia chromosome - blood looks like marrow
Varying shapes
CML
Hodgkin
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

30. What does CRAB stand for in multiple myeloma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
EBV
Schistocytes and inc LDH
Produce antibody - lots of RER and golgi

31. What are the variants of the RS cells
Incactivates II - VII - IX - X - XI - XII
Lacunar in nodular sclerosing variant
G6PD
Mantle cell lymphoma

32. Serum iron - transferrin - ferritin lab values for pregs - OCP use
G6PD
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Fibrinogen bind GpIIb/IIIa and links platelts
Nl - up - nl

33. What is the affected enzyme in acute intermittment porphyria
Exposed collagen upon endothelial damage
Africa = Jaw lesion - US = pelvis or abdomen
Porphobilinogen - delta ALA - uroporphyrin
Inc HbF and dec HbS

34. Deficiency in vWF

35. What do labs show in DIC?
Inc HbF and dec HbS
DIC
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

36. What are the four levels of alpha thal?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
ATIII def
Eleveated PT - PTT
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

37. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
Inducers of primary antibody response
Inc vasodiltion - inc perm - inc pain
Aplastic anemia - pancytopenia

38. Fava beans - sufla drugs - infectinos
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Ringed sideronblasts with iron laden mitochondria
Examples of oxidative stress
Heinz bodies - seen in alpha thal and G6PD

39. What are the main associations with multiple myeloma?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
DIC - TTP/HUS - traumatic hemolysis
No lytic bone lesions in WM
Inc HbF and dec HbS

40. What are some classic examples of extravascular hemolysis
ADP and Ca
Hereditary spherocytosis - G6PD - sickle cell
Varying shapes
Lysosomes

41. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
Birbeck granules
Bone marrow infiltration - myelofibrosis
All trans retinoic acid

42. What portion of IgE can mast cells bind
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Fc
Acts to stabilize platelet plug

43. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Von Willebrand's disease
They bind vWF via GpIb
Fibrinogen bind GpIIb/IIIa and links platelts

44. What is the Ddx for a normocytic - normochromic anemia?
Dec plasma volume
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
MHC II and Fc receptors
MGUS - monoclonal gammopathy of undetermined significance

45. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
90% anearobically from glucose to lactate - 10% from HMP shunt
Bernard soulier
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Valine for glutamate

46. Bone pain plus anemia
Eleveated PT - PTT
Multiple myeloma
Polycythemia vera
Extrinsic - I - II - V - VII and X

47. Macro - ovalocyte
Gp1b
Abciximab
Protoporphyrin
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

48. Upregulated growth of leukocytes in bone marro
Reed - sternberg cells
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Leukemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

49. What is the receptor for fibrinogen?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
GpIIb/IIIa
B6
Factor V Leiden

50. What is the characteristic finding for MAHA on peripheral smear?
Produce antibody - lots of RER and golgi
Degrades fibrin mesh and converts C3 to C3a
Dendritic cells?
Schistocytes - helmet cells