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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. t(15;17)
Porphyrias
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
M3 AML (acute promyelocytic leukemia)
Bone marrow - thymus - blood (80% of circulating lymphos are T)

2. Can B cells function as APCs?
Langerhans cells
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Yes - via MHC II
Incactivates II - VII - IX - X - XI - XII

3. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Blistering cutaneous photosens - most common porphyria
Round densly staining nucleus with a small amount of pale cytoplasm
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
VWD

4. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Off center nuclues - clock face chromatin
Porphobilinogen - delta ALA - uroporphyrin
Protein C or S deficiency
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

5. What is the affected enzyme in acute intermittment porphyria
Positive osmootic fragility test and splenectomy
90% anearobically from glucose to lactate - 10% from HMP shunt
Porphobilinogen - delta ALA - uroporphyrin
Valine for glutamate

6. What do you see in peripheral smear in a pt with G6PD?
Africa = Jaw lesion - US = pelvis or abdomen
Beta 4
Bite cells and Heinz bodies
120 days

7. Why does B12 def cause neuro sx?
Histamine - heparin - and eosinohil chemotactic factors
IgG - Warm is GREAT
Examples of oxidative stress
B12 in fatty acid pathways leads to subacute combined degeneration

8. Ringed sideroblasts
Round densly staining nucleus with a small amount of pale cytoplasm
Sideroblastic anemia
Aplastic anemia - pancytopenia
Plasma cell

9. What does decreasing heme do to ALA synthase activity?
CD15 and CD30 pos
Peroxidase
ADP and Ca
Inc

10. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Idiopathic thrombocytopenia (ITP)
ATIII def
HIV or immunosupression
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

11. What substance accumulates in porphyria cutanea
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Uroporphyrin (tea colored urine)
Indirect coombs - agglutinate if serum anti RBC surface Ig

12. Basophilic nuclear remnants fonud in RBCs
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 -
Blasts > 5%
Howell - Jolly bodies - hypo/asplenia
Splicing sites and promotor sequences

13. What are the azuraphilic granules in PMNs
Uroporphyrinogen decarboxylase
Lysosomes
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Schistocytes - helmet cells

14. S-100 and CD1a with birbeck granules
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
All trans retinoic acid
Sideroblastic anemia
Langerhans cell histiocytosis

15. universal donor
No antigen - both antibodies
Lung disease - congenital heart diseaes - and high altitude
Hodgkin
TXA2 - dec blood flow - inc platelet aggregation

16. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Lead - and EtOH
VWF carries/protects factor VIII
Liver disease - abetalipoproteinemia - acntho = spiny
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

17. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Porphyrias
CLL (SLL without the peripheral lymphocytosis
Hereditary spherocytosis - G6PD - sickle cell

18. Which infections can cause MAHA?
Malaria - Babesia
Africa = Jaw lesion - US = pelvis or abdomen
Protein S - cleaves and invactivates Va and VIIIa
Cromolyn sodium

19. After an injury - What does vWF bind to begin platelet plug formation?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Exposed collagen upon endothelial damage
Mantle cell lymphoma
Contiguous

20. What do the iron studies show in sideroblastic anemia
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Extravascular
Inc serum iron - normal TIBC - inc ferratin
VIII

21. Eosinophils are highly phagocytic For what kind of complex?
Dec plasma volume
Antigen - antibody
Low in CML
VWF carries/protects factor VIII

22. What does Vit K deficiency cause?
Lysosomes
Dec synthesis of factors 1972 - protein C/S
Complications of sickle cell anemia
Signs and sx of aplastic anemia

23. Who are the professional APCs?
B12/folate def
Porphobilinogen - delta ALA - uroporphyrin
Inducers of primary antibody response
Dendritic cells?

24. What is the mutation in HbC
Low in CML
ADP and Ca
Lysine for glutamate at position 6
SLE - CLL - alpha methyldopa

25. Is HS extravascular or intravascular?
Fibrinogen
Reed - sternberg cells
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Extravascular

26. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
CML
Macrohemorrhage - hemarthroses - easy bruising
T(12;21)

27. What does NAACP stand for - in regards to the causes of eosinophiia?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Mantle cell - older males
DIC
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

28. anti - Ig antibody added to patients RBCs;
Blistering cutaneous photosens - most common porphyria
Inactivates it
Direct coombs - agglutinate if RBCs are coated with Ig
90% anearobically from glucose to lactate - 10% from HMP shunt

29. What is relative polycythemia
Prothrombin gene mutation
Ticlopidine/clopidogrel
Dec plasma volume
Protoporphyrin

30. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Pyruvate kinase def - extravascular
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Complications of sickle cell anemia
Signs and sx of aplastic anemia

31. What is a blast crisis
Von Willebrand's disease
Off center nuclues - clock face chromatin
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
CML to AML or All

32. What is the ddx for aplastic anemia

33. What symptoms are associated with Hodgkin lymphoma
B symptoms - fever night sweats - weight loss
Peroxidase
Splicing sites and promotor sequences
All

34. What is appropriate absolyte polycythemia associated with
Lung disease - congenital heart diseaes - and high altitude
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Her next Rh+ fetus
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 -

35. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
TXA2 - dec blood flow - inc platelet aggregation
Up - down - nl
Intrinsic - all factors except - VII - XIII

36. Conditions of defective heme synthesis leading to accumulation of heme precurors
2 to 10
Porphyrias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Inactivates it

37. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Africa = Jaw lesion - US = pelvis or abdomen
Def in factor IX
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

38. hypocellular bone marrown tih fatty infiltration
B6
Aplastic anemia - pancytopenia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Bernard soulier

39. How are the nucleus and the cytoplasm characterized for lymphocytes
Multiple myeloma
Round densly staining nucleus with a small amount of pale cytoplasm
Yes - via MHC II
Thrombotic thrombocytopenic purpura

40. What is the pathogenesis of sickle cell
Pyruvate kinase def - extravascular
Petechiae
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Sickle cell anemia

41. What are some causes of warm agglutinin autoimmune hemolytic anemia?
All trans retinoic acid
Splicing sites and promotor sequences
Paraprotein spike - monoclonal protein
SLE - CLL - alpha methyldopa

42. What are the extrinsic hemolytic normocytic anemias?
SLE - CLL - alpha methyldopa
...
Up - down - up
Fe def - thal - pb poison - sideroblastic anemia

43. What is the life spance of a platelet?
Multiple myeloma
8-10 days
Fc
Fibrinogen bind GpIIb/IIIa and links platelts

44. What is the receptor for vWF
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Gp1b
Positive osmootic fragility test and splenectomy

45. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Langerhans cell histiocytosis
Factor V Leiden
ATIII def
Def in factor IX

46. What is the tx for aplastic anemia
Factor V Leiden
Up - down - nl
Examples of oxidative stress
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

47. What is the pathogenesis of ACD?
90% anearobically from glucose to lactate - 10% from HMP shunt
Hb Barts - gamma4 in defect in all 4 alpha genes
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

48. What is the main source of energy in RBCs
Inc serum iron - normal TIBC - inc ferratin
90% anearobically from glucose to lactate - 10% from HMP shunt
Essential thrombocytosis
Lead - and EtOH

49. Which substrates begin the heme synthesis pathway
Helps platelts adhere to endothelium
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
DIC
Glycine and succinyl - coa

50. What is the defect in beta thal?
Glanzmann's throbmasthenia
Megakaryocytes
Ticlopidine/clopidogrel
Splicing sites and promotor sequences