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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. Sickle cell
Sickle cell anemia
Gp1b
Petechiae
Valine for glutamate

2. What percentage of WBCs are eosinophils?
Kids= exposure to lead paint - adults = battery - ammunition factory
VWD
Hereditary spherocytosis - G6PD - sickle cell
1 to 6

3. t(11;14)
Epoxide reductase - warfarin inhibits
Mantle cell lymphoma
Imatinib - anti bcr abl
Pyruvate kinase def - extravascular

4. What is a metabolic disorder tht can cause macrocytic anemia?
Orotic aciduria
Ankryin - band 4.1 or spectrin
Hydroxyurea - bone marrow transplant
Lymphoma

5. Bite cell
Beta thal
Aplastic anemia - pancytopenia
Burkitt lymphoma
G6PD

6. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Imatinib - anti bcr abl
TPA - cleavage of fibrin mesh
Round densly staining nucleus with a small amount of pale cytoplasm

7. What is the mutation in HbC
Paroxysmal nocturnal hemoglobinuria
Dec
Lysine for glutamate at position 6
...

8. What is the accumulated substance in lead poisoning
Mycosis fundgoides/Sezary syndrome
CD15 and CD30 pos
90% anearobically from glucose to lactate - 10% from HMP shunt
Protoporphyrin (blood)

9. what proteins can be defective in HS?
Mycosis fundgoides/Sezary syndrome
Anemia of chronic disease - Aplastic anemia - kidney disease
Ankryin - band 4.1 or spectrin
Nodular sclerosing

10. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
Valine for glutamate
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Antigen - antibody

11. What are the extrinsic hemolytic normocytic anemias?
Fc
Prothrombin gene mutation
...
Sideroblastic anemia

12. What is contained within the azurophilic granules of PMNs
Paraprotein spike - monoclonal protein
Beta chain underproduced - asymptomatic - heterozygote
Gp1b
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

13. From what cells are platelets derived from
Megakaryocytes
Bimodal - men>women - except for nodular sclerosing type
Lysosomes
120 days

14. What do the labs show for TTP?
VWF carries/protects factor VIII
Schistocytes and inc LDH
Hereditary spherocytosis - autoimmune hemolysis
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

15. Wilm's tumor - RCC - HCC - hydronephrosis
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Inc serum iron - normal TIBC - inc ferratin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

16. What is the enzyme defect in Porphyria cutanea tarda
B symptoms - fever night sweats - weight loss
Uroporphyrinogen decarboxylase
Cromolyn sodium
Inactivates it

17. What is the activation stage of platelet plug formation?
Antigen - antibody
B12 in fatty acid pathways leads to subacute combined degeneration
Anemia of chronic disease - Aplastic anemia - kidney disease
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

18. inc LDH - jaundice
Glycine and succinyl - coa
Extravascular hemolysis
DIC
Anti Rh

19. Which infections can cause MAHA?
Malaria - Babesia
Tissue
CML to AML or All
Spleen

20. deficiency in ADAMTS13 leading to dec degradation of vWF
Thrombotic thrombocytopenic purpura
Ankryin - band 4.1 or spectrin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

21. What virus can cause an aplastic crisis in pts with HS?
No platelet clumping
Birbeck granules
Antigen - antibody
Parvovirus

22. What are some classic examples of extravascular hemolysis
Aspirin
Hereditary spherocytosis - G6PD - sickle cell
Antigen - antibody
Up - down - up

23. What is the mutation in HbS
ATIII def
Valine for glutamate
Imatinib - anti bcr abl
Pyruvate kinase def - extravascular

24. Acanthocyte (spur cell)
Intravascular
No
Liver disease - abetalipoproteinemia - acntho = spiny
Blod - tissue - MACS

25. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Lacunar in nodular sclerosing variant
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Pos
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

26. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Langerhans cells
Round densly staining nucleus with a small amount of pale cytoplasm
Howell - Jolly bodies - hypo/asplenia

27. HTLV-1
8-10 days
Adult T cell lymphoma - presents with cutaneous lesions
DIC
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

28. What is the philadelphia chromosome
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
T(9;22) bcr abl
CD15 and CD30 pos
Inactivates it

29. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Up - down - nl
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Down - down - up
Round densly staining nucleus with a small amount of pale cytoplasm

30. anti - Ig antibody added to patients RBCs;
Inc HbA2 on electrophoresis
Direct coombs - agglutinate if RBCs are coated with Ig
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
VWD

31. What converts plasminogen to plasm and What does plasmin do?
Inc RBC - dec O2 sat - inc EPO
Blod - tissue - MACS
TPA - cleavage of fibrin mesh
Deficiency in factor VIII

32. What is appropriate absolute polycythemia
Inc RBC - dec O2 sat - inc EPO
Ferrocheletase and ALA dehydrogenase
Nodular sclerosing
Follicular lymphoma - indolent course

33. Serum iron - transferrin - ferritin lab values for hemochromatosis
Her next Rh+ fetus
Up - down - up
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

34. How are the nucleus and the cytoplasm characterized for lymphocytes
Multiple myeloma
5- FU - AZT - hydroxyurea
Round densly staining nucleus with a small amount of pale cytoplasm
Sideroblastic anemia

35. What is the Ddx for a normocytic - normochromic anemia?
Her next Rh+ fetus
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Extravascular

36. What state is commonly associated with nonHod lymphoma
Histamine - heparin - and eosinohil chemotactic factors
Up - down - nl
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
HIV or immunosupression

37. Which pathway and factorrs are tested by the PT coag test
Fc
Up - down - up
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Extrinsic - I - II - V - VII and X

38. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
Acts to stabilize platelet plug
Protein S - cleaves and invactivates Va and VIIIa
Myelofibrosis (marow is crying being its fibrosed'

39. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Dec plasma volume
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
G6PD
Histaminase and arylsulfatase

40. Where do you see hypersegmented polys?
Bimodal - men>women - except for nodular sclerosing type
B12/folate def
Glycine and succinyl - coa
AML

41. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Anti Rh
No antigen - both antibodies
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
IFN gama

42. What is the receptor for fibrinogen?
Follicular lymphoma - indolent course
GpIIb/IIIa
Varying sizes
AB - no antibodies

43. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Induces differentiation of myeloblasts
Essential thrombocytosis
Alpha thal - asian and african american
IFN gama

44. What are the four levels of alpha thal?
Fibrinogen bind GpIIb/IIIa and links platelts
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Glucose and heme - which inhibit ALA synthase

45. t(15;17)
Common a few days after oxidative stress in a pt with G6PD
Follicular lymphoma - indolent course
M3 AML (acute promyelocytic leukemia)
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

46. philadelphia chromosome - blood looks like marrow
CML
Africa = Jaw lesion - US = pelvis or abdomen
Peroxidase
Orotic aciduria

47. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
B12 def
B12/folate def

48. What does ADP do?
Sheets of lymphocytes interspersed with macs
VWF and fibrinogen
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)

49. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Up - down - nl
Bite cells and Heinz bodies

50. After an injury - What does vWF bind to begin platelet plug formation?
Exposed collagen upon endothelial damage
TPA - cleavage of fibrin mesh
DIC
Inhibits thrombin - ixa - xa - xiia - and activated by heparin