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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 is monoclonal expansion without symptoms associated with multiple myeloma?
Valine for glutamate
MGUS - monoclonal gammopathy of undetermined significance
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Ewing sarcoma

2. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Spleen
CLL (SLL without the peripheral lymphocytosis
MHC II and Fc receptors
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

3. universal recipient
Bite cells and Heinz bodies
AB - no antibodies
Signs and sx of aplastic anemia
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

4. Drug that inhibits COX and therefore TXA2 synthesis
Inc UCB
Langerhans cell histiocytosis
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Aspirin

5. What is the enzyme defect in Porphyria cutanea tarda
They bind vWF via GpIb
Uroporphyrinogen decarboxylase
Exposed collagen upon endothelial damage
Her next Rh+ fetus

6. hypocellular bone marrown tih fatty infiltration
Lysine for glutamate at position 6
Intravascular
Histaminase and arylsulfatase
Aplastic anemia - pancytopenia

7. What is the affected enzyme in acute intermittment porphyria
Essential thrombocytosis
Porphobilinogen - delta ALA - uroporphyrin
G6PD
Birbeck granules

8. What symptoms are associated with Hodgkin lymphoma
Prothrombin gene mutation
G6PD
B symptoms - fever night sweats - weight loss
Sickle cell

9. What are the likely exposures of kids and adults for lead poisoning
40 to 75
ADP and Ca
Kids= exposure to lead paint - adults = battery - ammunition factory
Ferrocheletase and ALA dehydrogenase

10. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Intravascular hemolysis
B12/folate def
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Acts to stabilize platelet plug

11. What does the large SA:volume ratio in RBCs help facilitate?
Extravascular hemolysis
Complications of sickle cell anemia
Easy gas exchange
Lysosomes

12. What percentage if WBCs are polys?
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
40 to 75
Fe def - thal - pb poison - sideroblastic anemia
G6PD

13. What is a blast crisis
Multiple myeloma
Leukemia
CML to AML or All
Round densly staining nucleus with a small amount of pale cytoplasm

14. What indicated a good prognosis in Hodgkin lymphoma?
T(12;21)
Complications of sickle cell anemia
Inc lymphoctes - and less RS cells
TRAP (tartrate resistant acid phosphatase

15. What is the tx for sickle cell
Thrombotic thrombocytopenic purpura
Multiple myeloma
Abciximab
Hydroxyurea - bone marrow transplant

16. What do the labs show for TTP?
...
HIV or immunosupression
Schistocytes and inc LDH
CML

17. How do platelet disorders present?
Varying shapes
Ankryin - band 4.1 or spectrin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Induces differentiation of myeloblasts

18. When is the peak incidence for nonHod lymphoma
Liver disease - abetalipoproteinemia - acntho = spiny
Leukemia
20 to 40
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

19. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Reed - sternberg cells
Polycythemia vera
Leukemia
Blood

20. Who are the professional APCs?
Alpha thal - asian and african american
Dendritic cells?
Dec plasma volume
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

21. Drug that inhbits the GpIIb/IIIa directly
Ringed sideronblasts with iron laden mitochondria
Alpha thal - asian and african american
Abciximab
Bernard soulier

22. Where are basophils found?
Histamine - heparin - and eosinohil chemotactic factors
Lymphoma
Heinz bodies - seen in alpha thal and G6PD
Blood

23. What is their role?
Mantle cell - older males
Thrombotic thrombocytopenic purpura
Inducers of primary antibody response
Burkitt lymphoma

24. What does LEAD stand for in lead poisoning?
Low O2 in papilla; can also get microhematuria from medullary infarcts
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 -
Ringed sideronblasts with iron laden mitochondria
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

25. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Idiopathic thrombocytopenia (ITP)
Sickle cell
Howell Jolly bodies

26. What does ADP do?
Valine for glutamate
Dec plasma volume
ATIII def
Helps platelts adhere to endothelium

27. Ddx for microcytic anemia
Tissue factor converst the VII and VIIa
Histamine - heparin - and eosinohil chemotactic factors
Fe def - thal - pb poison - sideroblastic anemia
Multiple myeloma

28. Wilm's tumor - RCC - HCC - hydronephrosis
Histaminase and arylsulfatase
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Pos
Adult T cell lymphoma - presents with cutaneous lesions

29. hemolytic in a newborn - dec ATP and rigid RBCs
Round densly staining nucleus with a small amount of pale cytoplasm
Lung disease - congenital heart diseaes - and high altitude
Uroporphyrinogen decarboxylase
Pyruvate kinase def - extravascular

30. Is G6PD intravascular or extravascular
Collagen - BM - activated platelets
Eleveated PT - PTT
Down - down - up
Intravascular

31. Which immunoglobulin is involved in warm agglutination?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Examples of oxidative stress
IgG - Warm is GREAT
Hodgkin

32. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
MAHA
Bone marrow infiltration - myelofibrosis
Exposed collagen upon endothelial damage

33. What reveresible things can a sideroblastic anemia
Lead - and EtOH
Inc HbA2 on electrophoresis
Beta chain underproduced - asymptomatic - heterozygote
Direct coombs - agglutinate if RBCs are coated with Ig

34. What is the receptor for vWF
Gp1b
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Indirect coombs - agglutinate if serum anti RBC surface Ig

35. How are the nucleus and the cytoplasm characterized for lymphocytes
Round densly staining nucleus with a small amount of pale cytoplasm
CML to AML or All
Helps platelts adhere to endothelium
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

36. Fibrotic obliteration of bone marow with teardrop cells

37. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
Beta 4
Folate def
Both alpha and beta thal

38. What is the life span of a normal RBC
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
120 days
Valine for glutamate
Kids= exposure to lead paint - adults = battery - ammunition factory

39. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
Langerhans cell histiocytosis
Helminth infections major basic protein
Tissue

40. What are the neuro sx of B12 def?
TXA2 - dec blood flow - inc platelet aggregation
B symptoms - fever night sweats - weight loss
CD15 and CD30 pos
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

41. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Schistocytes and inc LDH
90% anearobically from glucose to lactate - 10% from HMP shunt
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

42. What do you see in peripheral smear in a pt with G6PD?
DIC
Leukemia
Bite cells and Heinz bodies
Inc UCB

43. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Hodgkin
Orotic aciduria
Dec

44. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Lysine for glutamate at position 6
Langerhans cell histiocytosis
Protein C or S deficiency
B12 def

45. What are the presenting symptoms of porphyria cutanea tarda
Hodgkin
Intrinsic - all factors except - VII - XIII
Blistering cutaneous photosens - most common porphyria
Mantle cell lymphoma

46. What state is commonly associated with nonHod lymphoma
Porphobilinogen - delta ALA - uroporphyrin
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
HIV or immunosupression
Bleeding - malnutrition/absorption - inc demand pregs

47. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
Anemia of chronic disease - Aplastic anemia - kidney disease
No antigen - both antibodies
HbSS

48. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Intravascular
CML to AML or All
Reed - sternberg cells
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

49. What portion of IgE can mast cells bind
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
8-10 days
Both alpha and beta thal
Fc

50. What is the tx for vWD
Sideroblastic anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Hereditary elloptocytosis
Malaria - Babesia