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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. Basophilica stippling
Fe def - thal - pb poison - sideroblastic anemia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Up - down - up
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

2. Sickle cell
Macrohemorrhage - hemarthroses - easy bruising
Helminth infections major basic protein
Sickle cell anemia
Hydroxyurea - bone marrow transplant

3. What causes hydrops fetalis
Round densly staining nucleus with a small amount of pale cytoplasm
Degrades fibrin mesh and converts C3 to C3a
Yes - via MHC II
Hb Barts - gamma4 in defect in all 4 alpha genes

4. What are the azuraphilic granules in PMNs
Lysosomes
Bite cells and Heinz bodies
Alpha thal - asian and african american
Glanzmann's throbmasthenia

5. What is their role?
VWF carries/protects factor VIII
Inducers of primary antibody response
Decrease EPO
Kids= exposure to lead paint - adults = battery - ammunition factory

6. Deficiency in vWF

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7. What are the anti aggregation factors?
Inc UCB
Sideroblastic anemia
Nl - up - nl
PGI2 - NO inc blood flow - dec platelet aggregation

8. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
Direct coombs - agglutinate if RBCs are coated with Ig
Nl PT - elevated PTT - intrinsic pathway defect
Sideroblastic anemia

9. What do the dense granules contain in platelets
Induces differentiation of myeloblasts
Liver disease - abetalipoproteinemia - acntho = spiny
Megakaryocytes
ADP and Ca

10. What finding you do you see in patients after splenectomy
90% anearobically from glucose to lactate - 10% from HMP shunt
Howell Jolly bodies
Glanzmann's throbmasthenia
Follicular lymphoma - indolent course

11. Which pathway and factorrs are tested by the PT coag test
Inc
Essential thrombocytosis
Extrinsic - I - II - V - VII and X
Burkitt lymphoma

12. What reveresible things can a sideroblastic anemia
B12 in fatty acid pathways leads to subacute combined degeneration
Lead - and EtOH
Inappropriate absolute with inc RBCs and EPO
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 -

13. Who has more severe disease - HbSS or HbSC
Induces differentiation of myeloblasts
DIC - TTP/HUS - traumatic hemolysis
HbSS
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

14. What is the activation stage of platelet plug formation?
Reed - sternberg cells
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Megakaryocytes
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

15. What is the treatment to prevent a woman from forming anti Rh antibody?
Inc HbF and dec HbS
Rhogam - Rh antigen immunoglobulin
VWF carries/protects factor VIII
No antigen - both antibodies

16. What is appropriate absolute polycythemia
Off center nuclues - clock face chromatin
Inc RBC - dec O2 sat - inc EPO
Dendritic cells?
Extravascular hemolysis

17. Elderly - mature b cell tumor with filamentous - hairlike projections
Yes - via MHC II
Fc
Hairy cell leukemia
B cells

18. What signal activates MACS
IFN gama
Low in CML
Abciximab
Lysosomes

19. What is the affected enzyme in lead poisoning
Ferrocheletase and ALA dehydrogenase
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Dendritic cells?
Kids= exposure to lead paint - adults = battery - ammunition factory

20. What is a blast crisis
A antigen and B antibodies
Factor V Leiden
CML to AML or All
Hereditary elloptocytosis

21. What is the ddx for aplastic anemia

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22. Drug that inhibits COX and therefore TXA2 synthesis
Folate def
Complications of sickle cell anemia
Aspirin
2 to 10

23. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Uroporphyrin (tea colored urine)
Beta thal
Varying shapes
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

24. philadelphia chromosome - blood looks like marrow
Uroporphyrin (tea colored urine)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Tissue factor converst the VII and VIIa
CML

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

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26. When do you see MAHA?
T(12;21)
They bind vWF via GpIb
Dec
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

27. t(15;17)
...
M3 AML (acute promyelocytic leukemia)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Epoxide reductase - warfarin inhibits

28. Ringed sideroblasts
CML
DIC
Hereditary spherocytosis - G6PD - sickle cell
Sideroblastic anemia

29. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Fibrinogen
Folate def
VWD
Prothrombin gene mutation

30. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
IgG - Warm is GREAT
B12 def
Helps platelts adhere to endothelium
Blod - tissue - MACS

31. What do the labs show for TTP?
Signs and sx of aplastic anemia
2 to 10
IgG - Warm is GREAT
Schistocytes and inc LDH

32. What is hemophiliia A
Deficiency in factor VIII
Gp1b
Plummer - vinson syndrome
2 to 10

33. What is the treatment for sideroblastic anemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Inc large vWF multimers - inc platelet aggregation and thrombosis
Low O2 in papilla; can also get microhematuria from medullary infarcts
B6 therapy (pyrodixine)

34. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Orotic aciduria
Deficiency in factor VIII

35. What is the defect in beta thal?
Splicing sites and promotor sequences
Inc vasodiltion - inc perm - inc pain
Intravascular hemolysis
Bleeding - malnutrition/absorption - inc demand pregs

36. What do the platelets bind? What is the step called
Anemia of chronic disease - Aplastic anemia - kidney disease
Extravascular
They bind vWF via GpIb
T(12;21)

37. How does vWD cause elevated PTT?
Lysine for glutamate at position 6
40 to 75
Abciximab
VWF carries/protects factor VIII

38. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Hodgkin
Dec plasma volume
Nodular sclerosing

39. What are the variants of the RS cells
Off center nuclues - clock face chromatin
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Incactivates II - VII - IX - X - XI - XII
Lacunar in nodular sclerosing variant

40. Which immunoglobulin is involved in warm agglutination?
IgG - Warm is GREAT
HIV or immunosupression
Rhogam - Rh antigen immunoglobulin
Porphyrias

41. Does CML have a JAK2 mut
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
No
Helps platelts adhere to endothelium
AB - no antibodies

42. Drug that inhbits the GpIIb/IIIa directly
HIV or immunosupression
Abciximab
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
MGUS - monoclonal gammopathy of undetermined significance

43. Who are the professional APCs?
Dendritic cells?
B antigena and A antibodes
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Intravascular hemolysis

44. How do platelet disorders present?
Both alpha and beta thal
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Valine for glutamate

45. What virus is associated with Burkitt lymphoma
Hereditary spherocytosis
Sideroblastic anemia
Up - down - up
EBV

46. Serum iron - transferrin - ferritin lab values for iron def anemia
Myelofibrosis (marow is crying being its fibrosed'
TXA2 - dec blood flow - inc platelet aggregation
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Down - up - down

47. What does antithrombin do and What activates it?
Idiopathic thrombocytopenia (ITP)
HIV or immunosupression
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Induces differentiation of myeloblasts

48. What does increasing heme do to ALA synthase activity
Dec
Inappropriate absolute with inc RBCs and EPO
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
20 to 40

49. Which drugs can cause macrocytic anemia?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
5- FU - AZT - hydroxyurea
Howell Jolly bodies
Birbeck granules

50. What substances are released in mast cell degranulation
Up - down - up
Hydroxyurea - bone marrow transplant
Blasts > 5%
Histamine - heparin - and eosinohil chemotactic factors