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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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1. Which substrates begin the heme synthesis pathway
Prothrombin gene mutation
Nl - up - nl
Varying shapes
Glycine and succinyl - coa

2. What is the Ddx for nonhemolytic normocytic anemia
Anemia of chronic disease - Aplastic anemia - kidney disease
MAHA
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

3. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Heinz bodies - seen in alpha thal and G6PD
G6PD
Uroporphyrin (tea colored urine)

4. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Histaminase and arylsulfatase
Lysosomes
Langerhans cell histiocytosis

5. What substance prevents mast cells degranulation?
Mycosis fundgoides/Sezary syndrome
Uroporphyrinogen decarboxylase
Cromolyn sodium
Common a few days after oxidative stress in a pt with G6PD

6. What do platelets release necessary for coagulation cascaed?
Plummer - vinson syndrome
Indirect coombs - agglutinate if serum anti RBC surface Ig
Burkitt lymphoma
ADP and Ca

7. What do they express on their surface?
B antigena and A antibodes
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 -
MHC II and Fc receptors
90% anearobically from glucose to lactate - 10% from HMP shunt

8. Ringed sideroblasts
Inducers of primary antibody response
Glucose and heme - which inhibit ALA synthase
Intrinsic - all factors except - VII - XIII
Sideroblastic anemia

9. What is the mutation in HbC
Histamine - heparin - and eosinohil chemotactic factors
Lysine for glutamate at position 6
Paroxysmal nocturnal hemoglobinuria
Diffuse Large b cell lymphoma

10. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Hb Barts - gamma4 in defect in all 4 alpha genes
Thrombotic thrombocytopenic purpura
No platelet clumping

11. What are the presenting symptoms of porphyria cutanea tarda
Multiple myeloma
Inducers of primary antibody response
Blistering cutaneous photosens - most common porphyria
B6

12. poikilocytosis
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
T(12;21)
VIII
Varying shapes

13. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
Hodgkin
B12 def
Factor V Leiden

14. What converts plasminogen to plasm and What does plasmin do?
Both alpha and beta thal
Anemia of chronic disease - Aplastic anemia - kidney disease
TPA - cleavage of fibrin mesh
...

15. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Paroxysmal nocturnal hemoglobinuria
Birbeck granules
Bone marrow infiltration - myelofibrosis

16. What vitamin is a cofactor for the first step of heme synthesis
Bleeding - malnutrition/absorption - inc demand pregs
Eleveated PT - PTT
B6
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

17. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Langerhans cells
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
5- FU - AZT - hydroxyurea

18. What is factor V leidin?

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19. hypocellular bone marrown tih fatty infiltration
Inc UCB
Acts to stabilize platelet plug
Aplastic anemia - pancytopenia
2 to 10

20. What is the effected enzyme in acute intermittment porphyria?
Mantle cell lymphoma
Ticlopidine/clopidogrel
Dendritic cells?
Porphobilinogen deaminase aka uroporphyrinogen I synthase

21. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Beta thal
Howell Jolly bodies
VWD

22. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Ferrocheletase and ALA dehydrogenase
Epoxide reductase - warfarin inhibits
Nl - up - nl
TRAP (tartrate resistant acid phosphatase

23. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
40 to 50
EBV
Orotic aciduria

24. What virus is associated with 50% of Hodgkin lymphoma
EBV
Blistering cutaneous photosens - most common porphyria
CML
Produce antibody - lots of RER and golgi

25. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Incactivates II - VII - IX - X - XI - XII
Howell Jolly bodies
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Down - down - up

26. What are the presenting symptoms of acute intermittent porphyria
B6
T(9;22) bcr abl
Nl - up - nl
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

27. After an injury - What does vWF bind to begin platelet plug formation?
Sideroblastic anemia
Ferrocheletase and ALA dehydrogenase
Positive osmootic fragility test and splenectomy
Exposed collagen upon endothelial damage

28. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
Decrease EPO
Howell Jolly bodies
Beta chain underproduced - asymptomatic - heterozygote

29. What are some classic examples of extravascular hemolysis
Round densly staining nucleus with a small amount of pale cytoplasm
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 -
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Hereditary spherocytosis - G6PD - sickle cell

30. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Round densly staining nucleus with a small amount of pale cytoplasm
Eleveated PT - PTT
Von Willebrand's disease
Beta thal

31. Fava beans - sufla drugs - infectinos
Fibrinogen bind GpIIb/IIIa and links platelts
Examples of oxidative stress
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Beta 4

32. What are the azuraphilic granules in PMNs
They bind vWF via GpIb
Lysosomes
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Protoporphyrin

33. What do platelets interact with to form a hemostatic plug
All
Fibrinogen
Dec synthesis of factors 1972 - protein C/S
Bernard soulier

34. What finding you do you see in patients after splenectomy
MAHA
Dec
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Howell Jolly bodies

35. discrete tumor masses arising from lymph nodes
Varying shapes
Lymphoma
Up - down - up
Hodgkin

36. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Schistocytes and inc LDH
Positive osmootic fragility test and splenectomy

37. In hemophilia A or B What do you see in the coag tests
Her next Rh+ fetus
HbSS
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Nl PT - elevated PTT - intrinsic pathway defect

38. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Complications of sickle cell anemia
DIC
T(9;22) bcr abl
Leukemoid reaction

39. What signal activates MACS
Hereditary spherocytosis - autoimmune hemolysis
IFN gama
Produce antibody - lots of RER and golgi
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

40. Deficiency in vWF

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41. What state is commonly associated with nonHod lymphoma
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
HIV or immunosupression
IFN gama
Abciximab

42. What does 'Neutrophils Like Making Everything Better' stand for?
Uroporphyrinogen decarboxylase
Inc lymphoctes - and less RS cells
Exposed collagen upon endothelial damage
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

43. Basophilic nuclear remnants fonud in RBCs
Howell - Jolly bodies - hypo/asplenia
CML
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Inducers of primary antibody response

44. What is appropriate absolyte polycythemia associated with
Lung disease - congenital heart diseaes - and high altitude
Multiple myeloma
B12 def
Both alpha and beta thal

45. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
No
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Leukemia
Parvovirus

46. What percentage of WBCs are eosinophils?
Spleen
1 to 6
Lymphoma
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

47. What is hemophiliia A
Deficiency in factor VIII
Adult T cell lymphoma - presents with cutaneous lesions
Leukemoid reaction
No

48. what proteins can be defective in HS?
Ankryin - band 4.1 or spectrin
Dec synthesis of factors 1972 - protein C/S
Protoporphyrin
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

49. What is the accumulated substance in lead poisoning
40 to 50
Petechiae
Tissue factor converst the VII and VIIa
Protoporphyrin (blood)

50. Megakaryocytosis
Heinz bodies - seen in alpha thal and G6PD
Essential thrombocytosis
VWF and fibrinogen
Petechiae