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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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1. How do platelet disorders present?
Down - down - up
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 -
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

2. What is the general pathology of a macrocytic anemia?
Easy gas exchange
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

3. What reveresible things can a sideroblastic anemia
Glucose and heme - which inhibit ALA synthase
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Lead - and EtOH
Direct coombs - agglutinate if RBCs are coated with Ig

4. What is the characteristic histologic finding in Hodgkin Lymphoma
Hereditary spherocytosis - G6PD - sickle cell
Reed - sternberg cells
Sideroblastic anemia
Spleen

5. What percentage if WBCs are polys?
VWF and fibrinogen
CML
Intravascular
40 to 75

6. Conditions of defective heme synthesis leading to accumulation of heme precurors
Def in factor IX
2 to 10
Porphyrias
B12/folate def

7. What does ectopic EPO produce
T(12;21)
Inappropriate absolute with inc RBCs and EPO
G6PD
Paraprotein spike - monoclonal protein

8. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Nodular sclerosing
Liver disease - abetalipoproteinemia - acntho = spiny
SLE - CLL - alpha methyldopa
VWD

9. inc LDH - jaundice
MGUS - monoclonal gammopathy of undetermined significance
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Extravascular hemolysis
Nl PT - elevated PTT - intrinsic pathway defect

10. What are the etiologies of B12 def
HbSS
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Uroporphyrin (tea colored urine)
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

11. What are the anti aggregation factors?
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
MAHA
PGI2 - NO inc blood flow - dec platelet aggregation
Def in factor IX

12. Plasma cell neoplasm
Hairy cell leukemia
Sideroblastic anemia
Extrinsic - I - II - V - VII and X
Multiple myeloma

13. What is HbH
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Aspirin
Beta 4
Aplastic anemia - pancytopenia

14. What is the most common nonhodgkin lymphoma
Yes - via MHC II
ATIII def
Sickle cell anemia
Diffuse Large b cell lymphoma

15. What is factor V leidin?

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16. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Inc serum iron - normal TIBC - inc ferratin
Mycosis fundgoides/Sezary syndrome
Positive osmootic fragility test and splenectomy
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

17. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
DDAVP (desmopressin) which releases stored vWF stored in endothelium
ATIII def
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

18. What do the platelets bind? What is the step called
They bind vWF via GpIb
ADP and Ca
Sideroblastic anemia
Ankryin - band 4.1 or spectrin

19. What signal activates MACS
Myelofibrosis (marow is crying being its fibrosed'
IFN gama
Acts to stabilize platelet plug
Tissue

20. Fava beans - sufla drugs - infectinos
Inc HbA2 on electrophoresis
Examples of oxidative stress
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Myelofibrosis (marow is crying being its fibrosed'

21. What is the effect of ACE on bradykinin
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inactivates it
Hereditary spherocytosis - G6PD - sickle cell
40 to 75

22. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Hb Barts - gamma4 in defect in all 4 alpha genes
Inc large vWF multimers - inc platelet aggregation and thrombosis
GpIIb/IIIa

23. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Fe def - thal - pb poison - sideroblastic anemia
B6
Pos

24. Ringed sideroblasts
Inc lymphoctes - and less RS cells
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 -
Sideroblastic anemia
No

25. Schisotcyte - helmet cell
DIC - TTP/HUS - traumatic hemolysis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Splicing sites and promotor sequences
Inc

26. Megakaryocytosis
Reed - sternberg cells
Essential thrombocytosis
Macrohemorrhage - hemarthroses - easy bruising
No lytic bone lesions in WM

27. What are the variants of the RS cells
Glycine and succinyl - coa
Blood
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Lacunar in nodular sclerosing variant

28. What are dendritic cells called in the skin?
Gp1b
Langerhans cells
Howell Jolly bodies
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

29. What is the are the presenting symptoms of lead poisoning in kids and adults
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Spleen
Beta 4
Inc UCB

30. Can B cells function as APCs?
Yes - via MHC II
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
EBV
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

31. What is contained within the azurophilic granules of PMNs
Antigen - antibody
Nodular sclerosing
Tissue factor converst the VII and VIIa
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

32. poikilocytosis
Abciximab
No antigen - both antibodies
Varying shapes
Beta thal

33. Adults present with cutaneous patches/nodules - indolent CD4+
Examples of oxidative stress
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Mycosis fundgoides/Sezary syndrome
Aspirin

34. What is appropriate absolyte polycythemia associated with
Intravascular
Lung disease - congenital heart diseaes - and high altitude
G6PD
Valine for glutamate

35. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
G6PD
Howell Jolly bodies
Paraprotein spike - monoclonal protein

36. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
Yes - via MHC II
ATIII def
TRAP (tartrate resistant acid phosphatase

37. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
GpIIb/IIIa
CLL (SLL without the peripheral lymphocytosis
5- FU - AZT - hydroxyurea
Porphobilinogen deaminase aka uroporphyrinogen I synthase

38. Who are the professional APCs?
Prothrombin gene mutation
Hodgkin
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Dendritic cells?

39. Which substrates begin the heme synthesis pathway
Porphobilinogen - delta ALA - uroporphyrin
Glycine and succinyl - coa
Prothrombin gene mutation
Down - up - down

40. What is the age breakdown for hodgkins
DIC
G6PD
Bimodal - men>women - except for nodular sclerosing type
Parvovirus

41. What is the characteristic finding for MAHA on peripheral smear?
T(12;21)
Varying sizes
Schistocytes - helmet cells
Ferrocheletase and ALA dehydrogenase

42. Deficiency in vWF

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43. What do they express on their surface?
Varying sizes
MHC II and Fc receptors
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Polycythemia vera

44. What do eosinophils defend against and What do they use to do it?
Helminth infections major basic protein
Beta thal
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
T(9;22) bcr abl

45. Serum iron - transferrin - ferritin lab values for hemochromatosis
Histamine - heparin - and eosinohil chemotactic factors
Up - down - up
AML
B12 in fatty acid pathways leads to subacute combined degeneration

46. Serum iron - transferrin - ferritin lab values for pregs - OCP use
TRAP (tartrate resistant acid phosphatase
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
40 to 75
Nl - up - nl

47. 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
A antigen and B antibodies
Hairy cell leukemia
Sickle cell
VIII

48. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
All
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Hb Barts - gamma4 in defect in all 4 alpha genes

49. Does CML have a JAK2 mut
No
Her next Rh+ fetus
Bimodal - men>women - except for nodular sclerosing type
Extravascular

50. Mutation in 3' untranslated region associated with venous clots
Factor V Leiden
Prothrombin gene mutation
ADP and Ca
Inc HbF and dec HbS