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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. Bite cell
Nodular sclerosing
G6PD
Langerhans cell histiocytosis
Porphobilinogen deaminase aka uroporphyrinogen I synthase

2. What are the etiologies of folate def
Protoporphyrin (blood)
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Leukemoid reaction

3. What percentage of WBCs are monocytes?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
2 to 10
Dendritic cells?
MAHA

4. What does LEAD stand for in lead poisoning?
Degrades fibrin mesh and converts C3 to C3a
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 -
Protein C or S deficiency
TPA - cleavage of fibrin mesh

5. What does bradykinin do?
Inc vasodiltion - inc perm - inc pain
Peroxidase
Inc RBC - dec O2 sat - inc EPO
They bind vWF via GpIb

6. Which infections can cause MAHA?
Malaria - Babesia
Sickle cell
Common a few days after oxidative stress in a pt with G6PD
B12 in fatty acid pathways leads to subacute combined degeneration

7. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
VWD
Blood
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sideroblastic anemia

8. Ddx for microcytic anemia
TPA - cleavage of fibrin mesh
Fe def - thal - pb poison - sideroblastic anemia
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Porphobilinogen deaminase aka uroporphyrinogen I synthase

9. How do platelet disorders present?
Multiple myeloma
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
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 -
Produce antibody - lots of RER and golgi

10. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Up - down - nl
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Polycythemia vera
Heinz bodies - seen in alpha thal and G6PD

11. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Fibrinogen
TXA2 - dec blood flow - inc platelet aggregation
No antigen - both antibodies

12. What symptoms are associated with Hodgkin lymphoma
Burkitt lymphoma
B symptoms - fever night sweats - weight loss
Protein C or S deficiency
Varying sizes

13. What virus is associated with Burkitt lymphoma
Inc RBC - dec O2 sat - inc EPO
ADP and Ca
B12 def
EBV

14. What is the Ddx for nonhemolytic normocytic anemia
Acts to stabilize platelet plug
Schistocytes and inc LDH
Anemia of chronic disease - Aplastic anemia - kidney disease
Parvovirus

15. Schisotcyte - helmet cell
Lysosomes
Sideroblastic anemia
DIC - TTP/HUS - traumatic hemolysis
Contiguous

16. Back pain - hemoglobinuria
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Common a few days after oxidative stress in a pt with G6PD
Blasts > 5%
Intrinsic - all factors except - VII - XIII

17. Megakaryocytosis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Essential thrombocytosis
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
DIC

18. poikilocytosis
Examples of oxidative stress
Varying shapes
Alpha thal - asian and african american
Idiopathic thrombocytopenia (ITP)

19. What are the extrinsic hemolytic normocytic anemias?
Paroxysmal nocturnal hemoglobinuria
...
Ringed sideronblasts with iron laden mitochondria
MHC II and Fc receptors

20. What do plasma cells do
Inactivates it
Inducers of primary antibody response
Rhogam - Rh antigen immunoglobulin
Produce antibody - lots of RER and golgi

21. What are the labs and tx for HS?
Pos
PGI2 - NO inc blood flow - dec platelet aggregation
Positive osmootic fragility test and splenectomy
Alpha thal - asian and african american

22. What does the large SA:volume ratio in RBCs help facilitate?
Paraprotein spike - monoclonal protein
Hereditary spherocytosis
Off center nuclues - clock face chromatin
Easy gas exchange

23. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
MAHA
Positive osmootic fragility test and splenectomy
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

24. What is the receptor for vWF
Gp1b
TPA - cleavage of fibrin mesh
Alpha thal - asian and african american
Megakaryocytes

25. What do platelets interact with to form a hemostatic plug
Fibrinogen
Glycine and succinyl - coa
Leukemoid reaction
5- FU - AZT - hydroxyurea

26. What do eosinophils defend against and What do they use to do it?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Helminth infections major basic protein
40 to 75
TXA2 - dec blood flow - inc platelet aggregation

27. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
G6PD
AB - no antibodies
Def in factor IX

28. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Glycine and succinyl - coa
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Intravascular

29. What does CRAB stand for in multiple myeloma
Dec
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Low O2 in papilla; can also get microhematuria from medullary infarcts

30. What is the therapy for the M3 variant?
Blasts > 5%
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
All trans retinoic acid
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

31. t(11:22)
Malaria - Babesia
Ewing sarcoma
Beta thal
Multiple myeloma

32. What are the age ranges for the various leukemias
Extravascular
VWF and fibrinogen
Peroxidase
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

33. Where are mast cells found
Myelofibrosis (marow is crying being its fibrosed'
CNS and testis
Factor V Leiden
Tissue

34. What is the Ddx for for a macrocytic anemia
DIC
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
SLE - CLL - alpha methyldopa

35. Where do B cells arise from - mature - and migrate to...
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
T(9;22) bcr abl

36. Does CML have a JAK2 mut
No
Easy gas exchange
Macrohemorrhage - hemarthroses - easy bruising
Deficiency in factor VIII

37. What do labs show in ITP?
Inc lymphoctes - and less RS cells
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Fibrinogen bind GpIIb/IIIa and links platelts
Hodgkin

38. What is the effected enzyme in acute intermittment porphyria?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Blod - tissue - MACS
All trans retinoic acid
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

39. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Lacunar in nodular sclerosing variant
No platelet clumping

40. Mutation in 3' untranslated region associated with venous clots
Parvovirus
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Prothrombin gene mutation
Hairy cell leukemia

41. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Fe def - thal - pb poison - sideroblastic anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
120 days
Yes - via MHC II

42. What is the aggregation phase of ppf?
Intrinsic - all factors except - VII - XIII
Bimodal - men>women - except for nodular sclerosing type
Bernard soulier
Fibrinogen bind GpIIb/IIIa and links platelts

43. Where are basophils found?
Glycine and succinyl - coa
Low in CML
Complications of sickle cell anemia
Blood

44. What is the pathogenesis of ACD?
Glanzmann's throbmasthenia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Protein S - cleaves and invactivates Va and VIIIa
Hairy cell leukemia

45. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
VWD
Inappropriate absolute with inc RBCs and EPO
Reed - sternberg cells

46. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Varying shapes
Inducers of primary antibody response
Spleen
Down - down - up

47. What are the azuraphilic granules in PMNs
Hydroxyurea - bone marrow transplant
Off center nuclues - clock face chromatin
Lysosomes
Plasma cell

48. bcl -2 t(14;18) adults
Paraprotein spike - monoclonal protein
Follicular lymphoma - indolent course
Her next Rh+ fetus
Protein C or S deficiency

49. What do the labs show for TTP?
Low in CML
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Schistocytes and inc LDH
5- FU - AZT - hydroxyurea

50. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
Orotic aciduria
Histamine - heparin - and eosinohil chemotactic factors
Yes - via MHC II