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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. What causes hydrops fetalis
Sheets of lymphocytes interspersed with macs
Hb Barts - gamma4 in defect in all 4 alpha genes
Megakaryocytes
Kids= exposure to lead paint - adults = battery - ammunition factory

2. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Collagen - BM - activated platelets
Inc HbF and dec HbS
Sickle cell
Intravascular hemolysis

3. Is HS extravascular or intravascular?
No antigen - both antibodies
Bleeding - malnutrition/absorption - inc demand pregs
Peroxidase
Extravascular

4. What percentage if WBCs are polys?
Sheets of lymphocytes interspersed with macs
MAHA
40 to 75
Low O2 in papilla; can also get microhematuria from medullary infarcts

5. What do plasma cells do
Produce antibody - lots of RER and golgi
EBV
Both alpha and beta thal
Dec plasma volume

6. What virus is associated with 50% of Hodgkin lymphoma
Aplastic anemia - pancytopenia
Glanzmann's throbmasthenia
EBV
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

7. Eosinophils are highly phagocytic For what kind of complex?
Leukemia
Porphobilinogen - delta ALA - uroporphyrin
Antigen - antibody
Protein C or S deficiency

8. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
Up - down - nl
Histamine - heparin - and eosinohil chemotactic factors
DIC - TTP/HUS - traumatic hemolysis

9. Where do you see hypersegmented polys?
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Porphyrias
Degrades fibrin mesh and converts C3 to C3a
B12/folate def

10. Where are basophils found?
2 to 10
Blood
Mantle cell lymphoma
Incactivates II - VII - IX - X - XI - XII

11. What is the tx for vWD
ADP and Ca
Beta 4
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
DDAVP (desmopressin) which releases stored vWF stored in endothelium

12. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
CLL (SLL without the peripheral lymphocytosis
Mantle cell lymphoma
Schistocytes and inc LDH

13. drug that inhibits ADP induced expression of GpIIb/IIIa
Blod - tissue - MACS
Protoporphyrin (blood)
Plasma cell
Ticlopidine/clopidogrel

14. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
All trans retinoic acid
Pos
Easy gas exchange
Hodgkin

15. What signal activates MACS
Howell - Jolly bodies - hypo/asplenia
IFN gama
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

16. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Hereditary spherocytosis
Heinz bodies - seen in alpha thal and G6PD
Langerhans cell histiocytosis
Examples of oxidative stress

17. Plasma cell neoplasm
Von Willebrand's disease
TRAP (tartrate resistant acid phosphatase
Def in factor IX
Multiple myeloma

18. What activates the intrinsic pathway?
B12/folate def
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Collagen - BM - activated platelets
Howell Jolly bodies

19. Why can newborns with sickle cell be asymptomatic
EBV
Helps platelts adhere to endothelium
Inc HbF and dec HbS
All trans retinoic acid

20. What are the anti aggregation factors?
PGI2 - NO inc blood flow - dec platelet aggregation
Glanzmann's throbmasthenia
Multiple myeloma
Africa = Jaw lesion - US = pelvis or abdomen

21. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Histaminase and arylsulfatase
Abciximab
Aspirin

22. What do labs show in DIC?
Ringed sideronblasts with iron laden mitochondria
Inducers of primary antibody response
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
...

23. What are the presenting symptoms of porphyria cutanea tarda
AML
Blistering cutaneous photosens - most common porphyria
Lysine for glutamate at position 6
Sideroblastic anemia

24. What is the pathogenesis of ACD?
Megakaryocytes
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Fibrinogen bind GpIIb/IIIa and links platelts

25. What do platelets interact with to form a hemostatic plug
Megakaryocytes
Inactivates it
VIII
Fibrinogen

26. What is the life spance of a platelet?
Myelofibrosis (marow is crying being its fibrosed'
Multiple myeloma
Anti Rh
8-10 days

27. What is the pathogenesis of aplastic anemia with kidney disease
Paraprotein spike - monoclonal protein
Decrease EPO
No
Sickle cell anemia

28. What is makes a leukemia acute
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Extravascular
Blasts > 5%
Inc large vWF multimers - inc platelet aggregation and thrombosis

29. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Up - down - nl
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Inc vasodiltion - inc perm - inc pain
Idiopathic thrombocytopenia (ITP)

30. What does Vit K deficiency cause?
Dec synthesis of factors 1972 - protein C/S
IgG - Warm is GREAT
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Common a few days after oxidative stress in a pt with G6PD

31. what proteins can be defective in HS?
Intravascular hemolysis
Ankryin - band 4.1 or spectrin
Tissue
Easy gas exchange

32. What converts plasminogen to plasm and What does plasmin do?
TPA - cleavage of fibrin mesh
Inappropriate absolute with inc RBCs and EPO
Hairy cell leukemia
MAHA

33. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
EBV
Aspirin
Epoxide reductase - warfarin inhibits
Anemia of chronic disease - Aplastic anemia - kidney disease

34. What is factor V leidin?

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35. What does antithrombin do and What activates it?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Anti Rh
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

36. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
B12 in fatty acid pathways leads to subacute combined degeneration
Her next Rh+ fetus
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Inc vasodiltion - inc perm - inc pain

37. What is the characteristic lab finding on electrophoresis
Bone marrow - thymus - blood (80% of circulating lymphos are T)
B symptoms - fever night sweats - weight loss
Paraprotein spike - monoclonal protein
Signs and sx of aplastic anemia

38. What does ADP do?
CML to AML or All
90% anearobically from glucose to lactate - 10% from HMP shunt
Helps platelts adhere to endothelium
Exposed collagen upon endothelial damage

39. What are some classic examples of extravascular hemolysis
Protoporphyrin
Hereditary spherocytosis - G6PD - sickle cell
Blod - tissue - MACS
20 to 40

40. Which pathway and factors are tested in the PTT test
Sickle cell
Malaria - Babesia
CNS and testis
Intrinsic - all factors except - VII - XIII

41. Target cell

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42. Who has more severe disease - HbSS or HbSC
Reed - sternberg cells
Petechiae
VWF carries/protects factor VIII
HbSS

43. After an injury - What does vWF bind to begin platelet plug formation?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
ADP and Ca
EBV
Exposed collagen upon endothelial damage

44. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Inappropriate absolute with inc RBCs and EPO
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Off center nuclues - clock face chromatin
Complications of sickle cell anemia

45. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Malaria - Babesia
Blistering cutaneous photosens - most common porphyria
VWF and fibrinogen
Leukemia

46. Bite cell
Inactivates it
G6PD
Antigen - antibody
Von Willebrand's disease

47. Ddx for microcytic anemia
EBV
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Fe def - thal - pb poison - sideroblastic anemia

48. Elderly - mature b cell tumor with filamentous - hairlike projections
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Off center nuclues - clock face chromatin
Lymphoma
Hairy cell leukemia

49. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
B6
Essential thrombocytosis
Intrinsic - all factors except - VII - XIII

50. Serum iron - transferrin - ferritin lab values for iron def anemia
Down - up - down
Glycine and succinyl - coa
MAHA
VIII