Test your basic knowledge |

Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
If you are not ready to take this test, you can study here.
Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.

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. Ringed sideroblasts
No lytic bone lesions in WM
Africa = Jaw lesion - US = pelvis or abdomen
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Sideroblastic anemia

2. What do eosinophils defend against and What do they use to do it?
Helminth infections major basic protein
Leukemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Ankryin - band 4.1 or spectrin

3. What vitamin is a cofactor for the first step of heme synthesis
Lung disease - congenital heart diseaes - and high altitude
Porphyrias
PGI2 - NO inc blood flow - dec platelet aggregation
B6

4. What is the general pathology of a macrocytic anemia?
Pos
Deficiency in factor VIII
Intravascular hemolysis
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

5. What are the variants of the RS cells
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Incactivates II - VII - IX - X - XI - XII
Lacunar in nodular sclerosing variant
Follicular lymphoma - indolent course

6. What are the pro aggregation factors?
Fc
Petechiae
Uroporphyrinogen decarboxylase
TXA2 - dec blood flow - inc platelet aggregation

7. What are the main associations with multiple myeloma?
G6PD
Uroporphyrin (tea colored urine)
Helps platelts adhere to endothelium
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

8. Basophilic nuclear remnants fonud in RBCs
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
MAHA
20 to 40
Howell - Jolly bodies - hypo/asplenia

9. What is the presenting scenario for TTP?
Porphyrias
Bimodal - men>women - except for nodular sclerosing type
MAHA
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

10. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
ATIII def
Lung disease - congenital heart diseaes - and high altitude
Epoxide reductase - warfarin inhibits
90% anearobically from glucose to lactate - 10% from HMP shunt

11. What do labs show in DIC?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
G6PD
Leukemoid reaction
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

12. What is monoclonal expansion without symptoms associated with multiple myeloma?
Leukemoid reaction
Hb Barts - gamma4 in defect in all 4 alpha genes
Hereditary spherocytosis
MGUS - monoclonal gammopathy of undetermined significance

13. What is a blast crisis
VWD
Sideroblastic anemia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
CML to AML or All

14. What do the platelets bind? What is the step called
They bind vWF via GpIb
Protein C or S deficiency
Fibrinogen
Nodular sclerosing

15. What percentage of WBCs are eosinophils?
1 to 6
Low in CML
Beta 4
Paroxysmal nocturnal hemoglobinuria

16. What cell is primarily involved in non Hod lymph
No lytic bone lesions in WM
IFN gama
Beta thal
B cells

17. What substance is Fe added to to yield heme
VWF carries/protects factor VIII
Lysosomes
Incactivates II - VII - IX - X - XI - XII
Protoporphyrin

18. What is appropriate absolyte polycythemia associated with
T(12;21)
Lung disease - congenital heart diseaes - and high altitude
Lysine for glutamate at position 6
Orotic aciduria

19. Where do you see hypersegmented polys?
120 days
B12/folate def
Common a few days after oxidative stress in a pt with G6PD
Hereditary spherocytosis

20. In hemophilia A or B What do you see in the coag tests
Dendritic cells?
Imatinib - anti bcr abl
Nl PT - elevated PTT - intrinsic pathway defect
Spleen

21. What virus is associated with Burkitt lymphoma
Down - up - down
Aspirin
EBV
120 days

22. What is the Ddx for a normocytic - normochromic anemia?
Langerhans cells
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Imatinib - anti bcr abl
B12/folate def

23. What are dendritic cells called in the skin?
VWD
Intrinsic - all factors except - VII - XIII
Langerhans cells
Megakaryocytes

24. What is the treatment for lead poising?
Pyruvate kinase def - extravascular
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Dec synthesis of factors 1972 - protein C/S
Beta chain underproduced - asymptomatic - heterozygote

25. What percentage of WBCs are monocytes?
B12 def
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
2 to 10
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

26. How does the therapy for M3 vairant work?
B6
Induces differentiation of myeloblasts
Hb Barts - gamma4 in defect in all 4 alpha genes
DIC

27. What signal activates MACS
IFN gama
Megakaryocytes
Varying shapes
Up - down - up

28. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
2 to 10
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Blistering cutaneous photosens - most common porphyria
All

29. What is the pathogenesis of sickle cell
Africa = Jaw lesion - US = pelvis or abdomen
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Abciximab

30. What does increasing heme do to ALA synthase activity
Dec
MHC II and Fc receptors
Inappropriate absolute with inc RBCs and EPO
Inc large vWF multimers - inc platelet aggregation and thrombosis

31. Spherocyte
Splicing sites and promotor sequences
Signs and sx of aplastic anemia
Hereditary spherocytosis - autoimmune hemolysis
TRAP (tartrate resistant acid phosphatase

32. universal recipient
AB - no antibodies
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

33. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Langerhans cell histiocytosis
TRAP (tartrate resistant acid phosphatase
Glycine and succinyl - coa

34. What reveresible things can a sideroblastic anemia
No platelet clumping
No lytic bone lesions in WM
Lead - and EtOH
Sideroblastic anemia

35. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Sickle cell
Down - up - down
Orotic aciduria

36. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
Hereditary spherocytosis - G6PD - sickle cell
Off center nuclues - clock face chromatin
TPA - cleavage of fibrin mesh

37. What substance prevents mast cells degranulation?
Induces differentiation of myeloblasts
Mantle cell lymphoma
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Cromolyn sodium

38. How are plasma cells characterized?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Valine for glutamate
Off center nuclues - clock face chromatin

39. What does bradykinin do?
T(9;22) bcr abl
Inc vasodiltion - inc perm - inc pain
Polycythemia vera
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

40. CD5+ - poor prognosis - t(11;14)
T(12;21)
40 to 50
Mantle cell - older males
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

41. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Common a few days after oxidative stress in a pt with G6PD
Essential thrombocytosis
Anti Rh
Down - down - up

42. Adults present with cutaneous patches/nodules - indolent CD4+
Leukemia
Folate def
Mycosis fundgoides/Sezary syndrome
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

43. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
B12 in fatty acid pathways leads to subacute combined degeneration
B cells
Dec plasma volume

44. Macro - ovalocyte
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

45. Which pathway and factors are tested in the PTT test
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Direct coombs - agglutinate if RBCs are coated with Ig
Intrinsic - all factors except - VII - XIII
Inc HbA2 on electrophoresis

46. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Plasma cell
Paroxysmal nocturnal hemoglobinuria
Reed - sternberg cells

47. What is the pattern of involvement and spread for nonHod lympho
Inc vasodiltion - inc perm - inc pain
Plasma cell
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Inc serum iron - normal TIBC - inc ferratin

48. What converts plasminogen to plasm and What does plasmin do?
All trans retinoic acid
Schistocytes and inc LDH
Examples of oxidative stress
TPA - cleavage of fibrin mesh

49. Which cell is neoplastic in multiple myeloma
Varying sizes
Round densly staining nucleus with a small amount of pale cytoplasm
EBV
Plasma cell

50. What are the extrinsic hemolytic normocytic anemias?
Produce antibody - lots of RER and golgi
...
Intravascular hemolysis
HbSS