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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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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. What are the four levels of alpha thal?
Off center nuclues - clock face chromatin
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Common a few days after oxidative stress in a pt with G6PD
Protoporphyrin (blood)

2. What cell is primarily involved in non Hod lymph
Histamine - heparin - and eosinohil chemotactic factors
B cells
MAHA
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

3. What is their role?
Direct coombs - agglutinate if RBCs are coated with Ig
Inducers of primary antibody response
AML
Nodular sclerosing

4. What does increasing heme do to ALA synthase activity
Dec
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Inc serum iron - normal TIBC - inc ferratin
A antigen and B antibodies

5. What is the coombs test results in AIHA?
Pos
Hairy cell leukemia
Gp1b
Bone marrow - thymus - blood (80% of circulating lymphos are T)

6. t(15;17)
DIC - TTP/HUS - traumatic hemolysis
M3 AML (acute promyelocytic leukemia)
Signs and sx of aplastic anemia
Plummer - vinson syndrome

7. Which infections can cause MAHA?
All trans retinoic acid
Malaria - Babesia
5- FU - AZT - hydroxyurea
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

8. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Fibrinogen bind GpIIb/IIIa and links platelts
Induces differentiation of myeloblasts
Pyruvate kinase def - extravascular

9. Basophilic nuclear remnants fonud in RBCs
Produce antibody - lots of RER and golgi
Howell - Jolly bodies - hypo/asplenia
Extravascular
Hereditary spherocytosis - G6PD - sickle cell

10. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Intravascular hemolysis
Her next Rh+ fetus
Lung disease - congenital heart diseaes - and high altitude
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

11. Which pathway and factorrs are tested by the PT coag test
T(12;21)
Extrinsic - I - II - V - VII and X
Protoporphyrin
VWD

12. What is the therapy for CML (philly chrom)
Imatinib - anti bcr abl
ADP and Ca
Essential thrombocytosis
IgG - Warm is GREAT

13. What is a blast crisis
Eleveated PT - PTT
DIC
Polycythemia vera
CML to AML or All

14. What causes the jaundice in extravascular hemolysis
Inc UCB
Tissue
T(12;21)
Inc HbA2 on electrophoresis

15. What is the pathogenesis of sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Lymphoma
CML to AML or All
Lysine for glutamate at position 6

16. iron deficiency anemia - esophageal web - atrophic glossitis
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Protoporphyrin (blood)
Deficiency in factor VIII
Plummer - vinson syndrome

17. What substance accumulates in porphyria cutanea
Inc RBC - dec O2 sat - inc EPO
Uroporphyrin (tea colored urine)
Extravascular hemolysis
CNS and testis

18. What does CRAB stand for in multiple myeloma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Splicing sites and promotor sequences

19. What do the iron studies show in sideroblastic anemia
TPA - cleavage of fibrin mesh
Inc serum iron - normal TIBC - inc ferratin
Lead - and EtOH
Lymphoma

20. What do platelets release necessary for coagulation cascaed?
Inc vasodiltion - inc perm - inc pain
ADP and Ca
EBV
Deficiency in factor VIII

21. Acanthocyte (spur cell)
GpIIb/IIIa
Tissue
Glanzmann's throbmasthenia
Liver disease - abetalipoproteinemia - acntho = spiny

22. How do platelet disorders present?
Down - up - down
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Aspirin
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

23. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
2 to 10
Histaminase and arylsulfatase
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Fibrinogen

24. Macro - ovalocyte
Up - down - up
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Lysine for glutamate at position 6

25. What percentage if WBCs are polys?
40 to 75
Fe def - thal - pb poison - sideroblastic anemia
Splicing sites and promotor sequences
Plasma cell

26. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Mycosis fundgoides/Sezary syndrome
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Fibrinogen bind GpIIb/IIIa and links platelts

27. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Valine for glutamate
IgG - Warm is GREAT
Inc HbF and dec HbS
Leukemoid reaction

28. Megakaryocytosis
Round densly staining nucleus with a small amount of pale cytoplasm
All
CML
Essential thrombocytosis

29. What is the general pathology of a macrocytic anemia?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Essential thrombocytosis
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

30. What vitamin is a cofactor for the first step of heme synthesis
B6
Porphobilinogen deaminase aka uroporphyrinogen I synthase
No antigen - both antibodies
Bernard soulier

31. Eosinophils are highly phagocytic For what kind of complex?
Beta chain underproduced - asymptomatic - heterozygote
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Antigen - antibody
VWD

32. t(8;14) c - myc gene
Burkitt lymphoma
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Bernard soulier
Pos

33. What portion of IgE can mast cells bind
Fc
Bimodal - men>women - except for nodular sclerosing type
Africa = Jaw lesion - US = pelvis or abdomen
Diffuse Large b cell lymphoma

34. What is the are the presenting symptoms of lead poisoning in kids and adults
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Birbeck granules
G6PD

35. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Splicing sites and promotor sequences
Inc serum iron - normal TIBC - inc ferratin
Histaminase and arylsulfatase
Blood

36. Back pain - hemoglobinuria
Ringed sideronblasts with iron laden mitochondria
Incactivates II - VII - IX - X - XI - XII
Common a few days after oxidative stress in a pt with G6PD
A antigen and B antibodies

37. What is the purpose of the fibrin mesh
Acts to stabilize platelet plug
Protein S - cleaves and invactivates Va and VIIIa
Lysine for glutamate at position 6
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

38. What causes the physiologic chloride shift and What does the chloride shift do?
...
Von Willebrand's disease
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Leukemia

39. What do eosinophils defend against and What do they use to do it?
Helminth infections major basic protein
Induces differentiation of myeloblasts
Low O2 in papilla; can also get microhematuria from medullary infarcts
Extravascular

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

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41. Conditions of defective heme synthesis leading to accumulation of heme precurors
Sideroblastic anemia
Hairy cell leukemia
Porphyrias
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

42. Is G6PD intravascular or extravascular
Deficiency in factor VIII
Dendritic cells?
Intravascular
Extrinsic - I - II - V - VII and X

43. What is the age breakdown for hodgkins
Sideroblastic anemia
Bimodal - men>women - except for nodular sclerosing type
Common a few days after oxidative stress in a pt with G6PD
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

44. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
AML
Splicing sites and promotor sequences
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
No lytic bone lesions in WM

45. What is appropriate absolute polycythemia
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Inc RBC - dec O2 sat - inc EPO
CML
No

46. philadelphia chromosome - blood looks like marrow
AML
CML
Lead - and EtOH
Intravascular hemolysis

47. What happens in beta thal major?
Inc
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
SLE - CLL - alpha methyldopa
Hydroxyurea - bone marrow transplant

48. What are the presenting symptoms of porphyria cutanea tarda
Hb Barts - gamma4 in defect in all 4 alpha genes
Blistering cutaneous photosens - most common porphyria
VWD
AML

49. Which substrates begin the heme synthesis pathway
CML
AB - no antibodies
Glycine and succinyl - coa
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

50. Ddx for microcytic anemia
Her next Rh+ fetus
B antigena and A antibodes
Fe def - thal - pb poison - sideroblastic anemia
Inc vasodiltion - inc perm - inc pain