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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. discrete tumor masses arising from lymph nodes
HbSS
Lymphoma
T(12;21)
Ticlopidine/clopidogrel

2. Deficiency in GpIb
Beta 4
Splicing sites and promotor sequences
Bernard soulier
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

3. What percentage if WBCs are polys?
Bone marrow infiltration - myelofibrosis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
40 to 75
Off center nuclues - clock face chromatin

4. hemolytic in a newborn - dec ATP and rigid RBCs
Indirect coombs - agglutinate if serum anti RBC surface Ig
Megakaryocytes
Inc lymphoctes - and less RS cells
Pyruvate kinase def - extravascular

5. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
No lytic bone lesions in WM
Cromolyn sodium
DIC - TTP/HUS - traumatic hemolysis

6. Is HS extravascular or intravascular?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Megakaryocytes
Extravascular
IgG - Warm is GREAT

7. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
A antigen and B antibodies
Leukemoid reaction
AML
HbSS

8. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Signs and sx of aplastic anemia
ADP and Ca

9. What is a blast crisis
CML to AML or All
Multiple myeloma
Factor V resistant to activated protein C's inhibition
EBV

10. In hemophilia A or B What do you see in the coag tests
No platelet clumping
40 to 50
Nl PT - elevated PTT - intrinsic pathway defect
CML

11. Bite cell
Mantle cell lymphoma
Helminth infections major basic protein
G6PD
IgG - Warm is GREAT

12. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
TRAP (tartrate resistant acid phosphatase
Antigen - antibody
Inc serum iron - normal TIBC - inc ferratin

13. Where do you see hypersegmented polys?
B12/folate def
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Ticlopidine/clopidogrel
Multiple myeloma

14. drug that inhibits ADP induced expression of GpIIb/IIIa
Splicing sites and promotor sequences
Ticlopidine/clopidogrel
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Ringed sideronblasts with iron laden mitochondria

15. What role does antithrombin play?
Lacunar in nodular sclerosing variant
Incactivates II - VII - IX - X - XI - XII
Dec plasma volume
Easy gas exchange

16. Where are mast cells found
Howell Jolly bodies
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Protein C or S deficiency
Tissue

17. What activates the intrinsic pathway?
Parvovirus
Def in factor IX
Collagen - BM - activated platelets
Down - up - down

18. What is contained within the azurophilic granules of PMNs
Nl - up - nl
Reed - sternberg cells
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

19. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Blood
Porphyrias
Helps platelts adhere to endothelium

20. What are the four levels of alpha thal?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Anti Rh

21. S-100 and CD1a with birbeck granules
Langerhans cell histiocytosis
Sideroblastic anemia
8-10 days
Idiopathic thrombocytopenia (ITP)

22. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Tissue factor converst the VII and VIIa
Helps platelts adhere to endothelium
Histaminase and arylsulfatase
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

23. What causes hydrops fetalis
Rhogam - Rh antigen immunoglobulin
Hb Barts - gamma4 in defect in all 4 alpha genes
Diffuse Large b cell lymphoma
Howell - Jolly bodies - hypo/asplenia

24. Which substrates begin the heme synthesis pathway
Inc vasodiltion - inc perm - inc pain
Glycine and succinyl - coa
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Hereditary spherocytosis - autoimmune hemolysis

25. What substances are released in mast cell degranulation
Histamine - heparin - and eosinohil chemotactic factors
Direct coombs - agglutinate if RBCs are coated with Ig
5- FU - AZT - hydroxyurea
Ferrocheletase and ALA dehydrogenase

26. What is the receptor for vWF
Inducers of primary antibody response
Gp1b
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Porphyrias

27. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

28. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Inc vasodiltion - inc perm - inc pain
Her next Rh+ fetus
Burkitt lymphoma

29. hypocellular bone marrown tih fatty infiltration
Aplastic anemia - pancytopenia
B antigena and A antibodes
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Schistocytes and inc LDH

30. What is the characteristic histologic finding in Hodgkin Lymphoma
No antigen - both antibodies
Aspirin
Reed - sternberg cells
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

31. t(15;17)
Essential thrombocytosis
Folate def
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
M3 AML (acute promyelocytic leukemia)

32. What is the difference of presentation of Burkitt in Africa vs the United States
Africa = Jaw lesion - US = pelvis or abdomen
Induces differentiation of myeloblasts
Protoporphyrin (blood)
DIC

33. What happens in betal thal minor?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Porphyrias
Beta chain underproduced - asymptomatic - heterozygote
Inc HbF and dec HbS

34. Serum iron - transferrin - ferritin lab values for iron def anemia
Down - up - down
Africa = Jaw lesion - US = pelvis or abdomen
No antigen - both antibodies
Incactivates II - VII - IX - X - XI - XII

35. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Porphyrias
No lytic bone lesions in WM
Fc
Complications of sickle cell anemia

36. What are the likely exposures of kids and adults for lead poisoning
Degrades fibrin mesh and converts C3 to C3a
Pyruvate kinase def - extravascular
AML
Kids= exposure to lead paint - adults = battery - ammunition factory

37. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
MGUS - monoclonal gammopathy of undetermined significance
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
ATIII def
B12 in fatty acid pathways leads to subacute combined degeneration

38. How does vWD cause elevated PTT?
B6
VWF carries/protects factor VIII
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Schistocytes - helmet cells

39. What substance prevents mast cells degranulation?
Eleveated PT - PTT
DIC
5- FU - AZT - hydroxyurea
Cromolyn sodium

40. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Inc UCB
VWF carries/protects factor VIII
Kids= exposure to lead paint - adults = battery - ammunition factory
Anti Rh

41. What the alpha granules contain in platelets?
VWF and fibrinogen
Indirect coombs - agglutinate if serum anti RBC surface Ig
AML
Examples of oxidative stress

42. Does CML have a JAK2 mut
No
8-10 days
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inappropriate absolute with inc RBCs and EPO

43. What is appropriate absolute polycythemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
B6
Inc RBC - dec O2 sat - inc EPO
Multiple myeloma

44. Target cell

45. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
Prothrombin gene mutation
Anti Rh
Dec plasma volume

46. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Bimodal - men>women - except for nodular sclerosing type
Liver disease - abetalipoproteinemia - acntho = spiny
SLE - CLL - alpha methyldopa
CLL (SLL without the peripheral lymphocytosis

47. What does NAACP stand for - in regards to the causes of eosinophiia?
CNS and testis
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Von Willebrand's disease
Contiguous

48. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Rhogam - Rh antigen immunoglobulin
Anti Rh
A antigen and B antibodies

49. Blood type B
Hereditary spherocytosis
B antigena and A antibodes
No lytic bone lesions in WM
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

50. What is HbH
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Sideroblastic anemia
Beta 4
Nl - up - nl