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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. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Ewing sarcoma
B cells
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

2. What is the difference of presentation of Burkitt in Africa vs the United States
Paroxysmal nocturnal hemoglobinuria
Plasma cell
Africa = Jaw lesion - US = pelvis or abdomen
Blod - tissue - MACS

3. What is the characteristic spread of Hodgkin Lymphoma
Low in CML
Signs and sx of aplastic anemia
Contiguous
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

4. Bite cell
Up - down - nl
EBV
G6PD
2 to 10

5. What is the tx for vWD
Fe def - thal - pb poison - sideroblastic anemia
G6PD
Down - down - up
DDAVP (desmopressin) which releases stored vWF stored in endothelium

6. Elderly - mature b cell tumor with filamentous - hairlike projections
ADP and Ca
Helminth infections major basic protein
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Hairy cell leukemia

7. What is the pathogenesis of TTP?
Intravascular
Inc large vWF multimers - inc platelet aggregation and thrombosis
B symptoms - fever night sweats - weight loss
PGI2 - NO inc blood flow - dec platelet aggregation

8. What do the dense granules contain in platelets
Plasma cell
Dec
Inc HbF and dec HbS
ADP and Ca

9. How is beta thal minor dx
Varying sizes
Inc HbA2 on electrophoresis
Helminth infections major basic protein
Degrades fibrin mesh and converts C3 to C3a

10. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
20 to 40
B6

11. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
...
Epoxide reductase - warfarin inhibits
90% anearobically from glucose to lactate - 10% from HMP shunt

12. What is the tx for sickle cell
Africa = Jaw lesion - US = pelvis or abdomen
Hydroxyurea - bone marrow transplant
Liver disease - abetalipoproteinemia - acntho = spiny
Birbeck granules

13. Is HS extravascular or intravascular?
Extravascular
Positive osmootic fragility test and splenectomy
Incactivates II - VII - IX - X - XI - XII
Anemia of chronic disease - Aplastic anemia - kidney disease

14. What are the etiologies of B12 def
Hereditary spherocytosis
Epoxide reductase - warfarin inhibits
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Malaria - Babesia

15. What is the mutation in HbC
Indirect coombs - agglutinate if serum anti RBC surface Ig
Lysine for glutamate at position 6
90% anearobically from glucose to lactate - 10% from HMP shunt
HbSS

16. Serum iron - transferrin - ferritin lab values for iron def anemia
B12 in fatty acid pathways leads to subacute combined degeneration
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Down - up - down

17. What is the pathogenesis of sickle cell
Essential thrombocytosis
GpIIb/IIIa
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Beta thal

18. Which drugs can cause macrocytic anemia?
Bleeding - malnutrition/absorption - inc demand pregs
Spleen
5- FU - AZT - hydroxyurea
Varying shapes

19. What reveresible things can a sideroblastic anemia
20 to 40
Fibrinogen bind GpIIb/IIIa and links platelts
Lead - and EtOH
Peroxidase

20. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Inactivates it
CNS and testis
Round densly staining nucleus with a small amount of pale cytoplasm

21. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Gp1b
Both alpha and beta thal
Leukemoid reaction
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 -

22. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
Degrades fibrin mesh and converts C3 to C3a
5- FU - AZT - hydroxyurea
G6PD

23. What is the affected enzyme in lead poisoning
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Decrease EPO
Dec synthesis of factors 1972 - protein C/S
Ferrocheletase and ALA dehydrogenase

24. What is the Ddx for nonhemolytic normocytic anemia
40 to 50
Myelofibrosis (marow is crying being its fibrosed'
IgG - Warm is GREAT
Anemia of chronic disease - Aplastic anemia - kidney disease

25. What does Vit K deficiency cause?
Bite cells and Heinz bodies
Dec synthesis of factors 1972 - protein C/S
1 to 6
Exposed collagen upon endothelial damage

26. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Beta thal
Anti Rh
B antigena and A antibodes

27. How are the nucleus and the cytoplasm characterized for lymphocytes
Leukemoid reaction
Beta 4
Round densly staining nucleus with a small amount of pale cytoplasm
Reed - sternberg cells

28. What is hemophilia B?
Def in factor IX
Bite cells and Heinz bodies
Protoporphyrin (blood)
Plasma cell

29. What is the pathogenesis of aplastic anemia with kidney disease
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
B12 in fatty acid pathways leads to subacute combined degeneration
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Decrease EPO

30. What do they express on their surface?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
MHC II and Fc receptors
Incactivates II - VII - IX - X - XI - XII
Sickle cell

31. After an injury - What does vWF bind to begin platelet plug formation?
CML
Exposed collagen upon endothelial damage
CLL (SLL without the peripheral lymphocytosis
Diffuse Large b cell lymphoma

32. What happens in beta thal major?
Up - down - up
Intrinsic - all factors except - VII - XIII
Incactivates II - VII - IX - X - XI - XII
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

33. universal donor
Produce antibody - lots of RER and golgi
PGI2 - NO inc blood flow - dec platelet aggregation
Fe def - thal - pb poison - sideroblastic anemia
No antigen - both antibodies

34. What do platelets interact with to form a hemostatic plug
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Fibrinogen
Protoporphyrin
All trans retinoic acid

35. What do the labs show for TTP?
Contiguous
Schistocytes and inc LDH
Protoporphyrin (blood)
Beta chain underproduced - asymptomatic - heterozygote

36. Ringed sideroblasts
A antigen and B antibodies
G6PD
Sideroblastic anemia
Aplastic anemia - pancytopenia

37. What virus can cause an aplastic crisis in pts with HS?
SLE - CLL - alpha methyldopa
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Parvovirus
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

38. Drug that inhibits COX and therefore TXA2 synthesis
Aspirin
B cells
Decrease EPO
Mycosis fundgoides/Sezary syndrome

39. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
B12/folate def
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Low in CML
HbSS

40. Blood type B
Megakaryocytes
1 to 6
B antigena and A antibodes
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

41. What are the four levels of alpha thal?
Protein S - cleaves and invactivates Va and VIIIa
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Follicular lymphoma - indolent course
Cromolyn sodium

42. What is the defect in beta thal?
Protein C or S deficiency
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
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 -
Splicing sites and promotor sequences

43. What does antithrombin do and What activates it?
...
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Diffuse Large b cell lymphoma

44. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
SLE - CLL - alpha methyldopa
Mantle cell lymphoma
Hydroxyurea - bone marrow transplant
Factor V Leiden

45. drug that inhibits ADP induced expression of GpIIb/IIIa
Inactivates it
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Ticlopidine/clopidogrel
Hodgkin

46. What does STOP Making New Thrombi stand for
EBV
Alpha thal - asian and african american
Kids= exposure to lead paint - adults = battery - ammunition factory
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

47. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
HbSS
Reed - sternberg cells
MGUS - monoclonal gammopathy of undetermined significance
All

48. What finding you do you see in patients after splenectomy
VWF carries/protects factor VIII
TRAP (tartrate resistant acid phosphatase
Howell Jolly bodies
Lung disease - congenital heart diseaes - and high altitude

49. What causes the physiologic chloride shift and What does the chloride shift do?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
B12 in fatty acid pathways leads to subacute combined degeneration
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Parvovirus

50. What is the characteristic lab finding on electrophoresis
Idiopathic thrombocytopenia (ITP)
Pos
Paraprotein spike - monoclonal protein
CML to AML or All