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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 do auer rods stain with
Africa = Jaw lesion - US = pelvis or abdomen
Peroxidase
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
CD15 and CD30 pos

2. What is the life span of a normal RBC
Her next Rh+ fetus
Tissue
SLE - CLL - alpha methyldopa
120 days

3. What are dendritic cells called in the skin?
Varying sizes
Down - up - down
Def in factor IX
Langerhans cells

4. When is the peak incidence for nonHod lymphoma
20 to 40
Inc large vWF multimers - inc platelet aggregation and thrombosis
CD15 and CD30 pos
...

5. What is the effect of ACE on bradykinin
GpIIb/IIIa
Inactivates it
Africa = Jaw lesion - US = pelvis or abdomen
Cromolyn sodium

6. What are the etiologies of folate def
Follicular lymphoma - indolent course
HbSS
Orotic aciduria
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

7. What do you see on peripheral smear with sideroblastic anemia
Dec plasma volume
CML
Histaminase and arylsulfatase
Ringed sideronblasts with iron laden mitochondria

8. bcl -2 t(14;18) adults
Mycosis fundgoides/Sezary syndrome
G6PD
Blood
Follicular lymphoma - indolent course

9. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Inc HbA2 on electrophoresis
All
Diffuse Large b cell lymphoma
Bone marrow - thymus - blood (80% of circulating lymphos are T)

10. hemolytic in a newborn - dec ATP and rigid RBCs
...
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Nodular sclerosing
Pyruvate kinase def - extravascular

11. What is their role?
Inc RBC - dec O2 sat - inc EPO
B12 in fatty acid pathways leads to subacute combined degeneration
Inducers of primary antibody response
MHC II and Fc receptors

12. What is the affected enzyme in acute intermittment porphyria
VWF carries/protects factor VIII
Porphobilinogen - delta ALA - uroporphyrin
CML
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

13. What is the characteristic lab finding on electrophoresis
TXA2 - dec blood flow - inc platelet aggregation
Paraprotein spike - monoclonal protein
Malaria - Babesia
Porphobilinogen - delta ALA - uroporphyrin

14. What are the neuro sx of B12 def?
Glycine and succinyl - coa
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Fc

15. What are the azuraphilic granules in PMNs
Hodgkin
Lysosomes
Macrohemorrhage - hemarthroses - easy bruising
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

16. What is hemophilia B?
Dec plasma volume
Def in factor IX
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Inc HbF and dec HbS

17. What are some classic examples of extravascular hemolysis
EBV
G6PD
Intravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell

18. What is the therapy for the M3 variant?
All trans retinoic acid
Essential thrombocytosis
Diffuse Large b cell lymphoma
Valine for glutamate

19. Where does All spread
Inc HbA2 on electrophoresis
Parvovirus
Langerhans cell histiocytosis
CNS and testis

20. universal donor
Lung disease - congenital heart diseaes - and high altitude
No antigen - both antibodies
Direct coombs - agglutinate if RBCs are coated with Ig
Leukemoid reaction

21. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Intravascular
MGUS - monoclonal gammopathy of undetermined significance
Inc serum iron - normal TIBC - inc ferratin
Sickle cell

22. What substance is Fe added to to yield heme
Hereditary spherocytosis - autoimmune hemolysis
Schistocytes - helmet cells
Protoporphyrin
Complications of sickle cell anemia

23. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
DIC
90% anearobically from glucose to lactate - 10% from HMP shunt
Inc UCB
Protein S - cleaves and invactivates Va and VIIIa

24. What does ectopic EPO produce
Decrease EPO
Abciximab
90% anearobically from glucose to lactate - 10% from HMP shunt
Inappropriate absolute with inc RBCs and EPO

25. Mutation in 3' untranslated region associated with venous clots
Ferrocheletase and ALA dehydrogenase
Prothrombin gene mutation
Inc UCB
Schistocytes - helmet cells

26. What is the difference of presentation of Burkitt in Africa vs the United States
Inc UCB
Africa = Jaw lesion - US = pelvis or abdomen
Plasma cell
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

27. What cell is primarily involved in non Hod lymph
B cells
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Ankryin - band 4.1 or spectrin
Leukemia

28. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Exposed collagen upon endothelial damage
B symptoms - fever night sweats - weight loss
Glanzmann's throbmasthenia

29. What do plasma cells do
Deficiency in factor VIII
Orotic aciduria
Produce antibody - lots of RER and golgi
Lysine for glutamate at position 6

30. What does STOP Making New Thrombi stand for
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Bernard soulier
8-10 days
Sideroblastic anemia

31. What is the tx for vWD
Both alpha and beta thal
Lead - and EtOH
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Produce antibody - lots of RER and golgi

32. What activates the intrinsic pathway?
Bone marrow infiltration - myelofibrosis
B12 def
Collagen - BM - activated platelets
Bimodal - men>women - except for nodular sclerosing type

33. What is the treatment for sideroblastic anemia
Liver disease - abetalipoproteinemia - acntho = spiny
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Africa = Jaw lesion - US = pelvis or abdomen
B6 therapy (pyrodixine)

34. What percentage of WBCs are monocytes?
2 to 10
Plummer - vinson syndrome
Folate def
Hereditary elloptocytosis

35. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Blasts > 5%
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Low in CML

36. Basophilica stippling
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
ADP and Ca
T(9;22) bcr abl
Plummer - vinson syndrome

37. What percentage if WBCs are polys?
Fc
Complications of sickle cell anemia
40 to 75
Pyruvate kinase def - extravascular

38. Which drugs can cause macrocytic anemia?
Valine for glutamate
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
5- FU - AZT - hydroxyurea

39. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
Hairy cell leukemia
G6PD
Blood

40. What is the Ddx for nonhemolytic normocytic anemia
Indirect coombs - agglutinate if serum anti RBC surface Ig
Anemia of chronic disease - Aplastic anemia - kidney disease
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Mantle cell - older males

41. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Examples of oxidative stress
B12/folate def
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

42. Causes of iron def?
Dendritic cells?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Bleeding - malnutrition/absorption - inc demand pregs
Lung disease - congenital heart diseaes - and high altitude

43. What is appropriate absolute polycythemia
Beta chain underproduced - asymptomatic - heterozygote
Inc RBC - dec O2 sat - inc EPO
1 to 6
Eleveated PT - PTT

44. What are the etiologies of B12 def
Sickle cell
Exposed collagen upon endothelial damage
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Porphyrias

45. What are the four levels of alpha thal?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Contiguous
Pos

46. Where do you see hypersegmented polys?
IFN gama
Schistocytes - helmet cells
B12/folate def
G6PD

47. What are the variants of the RS cells
Aspirin
Lacunar in nodular sclerosing variant
Antigen - antibody
Bimodal - men>women - except for nodular sclerosing type

48. Deficiency in vWF

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49. Plasma cell neoplasm
Multiple myeloma
MGUS - monoclonal gammopathy of undetermined significance
Protoporphyrin
Degrades fibrin mesh and converts C3 to C3a

50. Where are monocytes typically found - where do they go - and What do the differentiate into?
Blod - tissue - MACS
Blasts > 5%
Bite cells and Heinz bodies
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT