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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 does plasmin do?
40 to 50
Inducers of primary antibody response
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Degrades fibrin mesh and converts C3 to C3a

2. Where does All spread
Porphyrias
CNS and testis
Liver disease - abetalipoproteinemia - acntho = spiny
Hodgkin

3. Who are the professional APCs?
AML
Malaria - Babesia
Dendritic cells?
Complications of sickle cell anemia

4. What portion of IgE can mast cells bind
Bone marrow - thymus - blood (80% of circulating lymphos are T)
IFN gama
DIC - TTP/HUS - traumatic hemolysis
Fc

5. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
HbSS
Up - down - nl

6. Fava beans - sufla drugs - infectinos
GpIIb/IIIa
Examples of oxidative stress
AB - no antibodies
Idiopathic thrombocytopenia (ITP)

7. What is the effected enzyme in acute intermittment porphyria?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Mycosis fundgoides/Sezary syndrome
VIII
Nl - up - nl

8. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
A antigen and B antibodies
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Protein C or S deficiency
...

9. What is the main source of energy in RBCs
GpIIb/IIIa
Pyruvate kinase def - extravascular
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
90% anearobically from glucose to lactate - 10% from HMP shunt

10. Why can newborns with sickle cell be asymptomatic
Inc HbF and dec HbS
Bimodal - men>women - except for nodular sclerosing type
Africa = Jaw lesion - US = pelvis or abdomen
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

11. What is the characteristic finding for MAHA on peripheral smear?
B12 def
Schistocytes - helmet cells
Intravascular
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

12. What vitamin is a cofactor for the first step of heme synthesis
Paraprotein spike - monoclonal protein
DIC
Hereditary elloptocytosis
B6

13. What is appropriate absolute polycythemia
Paraprotein spike - monoclonal protein
Deficiency in factor VIII
Inc RBC - dec O2 sat - inc EPO
Follicular lymphoma - indolent course

14. What does STOP Making New Thrombi stand for
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Inc HbF and dec HbS
Deficiency in factor VIII

15. What is the characteristic spread of Hodgkin Lymphoma
Exposed collagen upon endothelial damage
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Contiguous
Beta thal

16. What is the receptor for fibrinogen?
GpIIb/IIIa
Howell - Jolly bodies - hypo/asplenia
ADP and Ca
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

17. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
Inc HbA2 on electrophoresis
Anemia of chronic disease - Aplastic anemia - kidney disease
VWF and fibrinogen

18. S-100 and CD1a with birbeck granules
Langerhans cell histiocytosis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Paraprotein spike - monoclonal protein
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

19. What causes the jaundice in extravascular hemolysis
Inc UCB
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Beta 4

20. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Gp1b
2 to 10
No

21. What does antithrombin do and What activates it?
Protein S - cleaves and invactivates Va and VIIIa
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Megakaryocytes
Ringed sideronblasts with iron laden mitochondria

22. CD5+ - poor prognosis - t(11;14)
HbSS
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Mantle cell - older males
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

23. Is HS extravascular or intravascular?
Positive osmootic fragility test and splenectomy
Extravascular
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

24. philadelphia chromosome - blood looks like marrow
Inappropriate absolute with inc RBCs and EPO
CML
Factor V Leiden
Up - down - up

25. iron deficiency anemia - esophageal web - atrophic glossitis
Plummer - vinson syndrome
They bind vWF via GpIb
Mycosis fundgoides/Sezary syndrome
Hereditary spherocytosis - autoimmune hemolysis

26. What is the presenting scenario for TTP?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Schistocytes and inc LDH
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

27. What are the four levels of alpha thal?
Hereditary spherocytosis
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Howell - Jolly bodies - hypo/asplenia
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

28. What are the main associations with multiple myeloma?
Fibrinogen bind GpIIb/IIIa and links platelts
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
ADP and Ca

29. What is the therapy for the M3 variant?
Anti Rh
B antigena and A antibodes
All trans retinoic acid
Bimodal - men>women - except for nodular sclerosing type

30. t(15;17)
Langerhans cell histiocytosis
Reed - sternberg cells
Blod - tissue - MACS
M3 AML (acute promyelocytic leukemia)

31. What is the most common nonhodgkin lymphoma
Acts to stabilize platelet plug
Hereditary elloptocytosis
Ticlopidine/clopidogrel
Diffuse Large b cell lymphoma

32. What virus is associated with Burkitt lymphoma
Lung disease - congenital heart diseaes - and high altitude
Inc HbF and dec HbS
Incactivates II - VII - IX - X - XI - XII
EBV

33. HTLV-1
Essential thrombocytosis
TPA - cleavage of fibrin mesh
Paroxysmal nocturnal hemoglobinuria
Adult T cell lymphoma - presents with cutaneous lesions

34. 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
Produce antibody - lots of RER and golgi
Helminth infections major basic protein
Uroporphyrin (tea colored urine)

35. What reveresible things can a sideroblastic anemia
B6
Lead - and EtOH
Tissue
Splicing sites and promotor sequences

36. Teardrop cell
40 to 75
Bone marrow infiltration - myelofibrosis
1 to 6
Blasts > 5%

37. Deficiency in GpIb
Bernard soulier
Inc HbA2 on electrophoresis
Malaria - Babesia
Blasts > 5%

38. What is the mutation in HbS
Valine for glutamate
VIII
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Hereditary elloptocytosis

39. What do you see in vit K def
Yes - via MHC II
Eleveated PT - PTT
Porphyrias
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

40. Schisotcyte - helmet cell
DIC - TTP/HUS - traumatic hemolysis
Abciximab
Thrombotic thrombocytopenic purpura
Inc serum iron - normal TIBC - inc ferratin

41. Ringed sideroblasts
Sideroblastic anemia
Helps platelts adhere to endothelium
Sheets of lymphocytes interspersed with macs
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

42. deficiency in ADAMTS13 leading to dec degradation of vWF
Round densly staining nucleus with a small amount of pale cytoplasm
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Thrombotic thrombocytopenic purpura
Idiopathic thrombocytopenia (ITP)

43. How is beta thal minor dx
Inc HbA2 on electrophoresis
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Hb Barts - gamma4 in defect in all 4 alpha genes

44. What substance is Fe added to to yield heme
Eleveated PT - PTT
Protoporphyrin
Leukemia
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

45. Why does B12 def cause neuro sx?
Lung disease - congenital heart diseaes - and high altitude
Multiple myeloma
B12 in fatty acid pathways leads to subacute combined degeneration
AML

46. What are some classic examples of extravascular hemolysis
EBV
Hereditary spherocytosis - G6PD - sickle cell
Reed - sternberg cells
Gp1b

47. What does the large SA:volume ratio in RBCs help facilitate?
Easy gas exchange
Dec synthesis of factors 1972 - protein C/S
Schistocytes - helmet cells
Ewing sarcoma

48. When do you see MAHA?
Hydroxyurea - bone marrow transplant
Inappropriate absolute with inc RBCs and EPO
Lymphoma
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

49. Where to T cells arise and mature - and migrate to...
Histaminase and arylsulfatase
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
SLE - CLL - alpha methyldopa

50. What are the presenting symptoms of acute intermittent porphyria
Hydroxyurea - bone marrow transplant
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inc HbF and dec HbS
Hereditary spherocytosis - G6PD - sickle cell