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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. What is the treatment for acute intermittent porphyria
Examples of oxidative stress
Glucose and heme - which inhibit ALA synthase
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

2. What is makes a leukemia acute
TPA - cleavage of fibrin mesh
Blasts > 5%
...
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

3. What is the coombs test results in AIHA?
Rhogam - Rh antigen immunoglobulin
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Complications of sickle cell anemia
Pos

4. What virus is associated with 50% of Hodgkin lymphoma
EBV
B symptoms - fever night sweats - weight loss
Helminth infections major basic protein
IFN gama

5. What are the extrinsic hemolytic normocytic anemias?
Extravascular
Rhogam - Rh antigen immunoglobulin
...
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

6. Deficiency in GpIb
Hereditary elloptocytosis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Bernard soulier
Ringed sideronblasts with iron laden mitochondria

7. What are the main associations with multiple myeloma?
MHC II and Fc receptors
Burkitt lymphoma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Fibrinogen bind GpIIb/IIIa and links platelts

8. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Multiple myeloma
SLE - CLL - alpha methyldopa
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Pyruvate kinase def - extravascular

9. What is a metabolic disorder tht can cause macrocytic anemia?
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Orotic aciduria
Down - up - down
Folate def

10. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Ankryin - band 4.1 or spectrin
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Complications of sickle cell anemia
Lysine for glutamate at position 6

11. Drug that inhibits COX and therefore TXA2 synthesis
Aspirin
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

12. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
Liver disease - abetalipoproteinemia - acntho = spiny
Malaria - Babesia
5- FU - AZT - hydroxyurea

13. What is the characteristic finding for MAHA on peripheral smear?
Mantle cell - older males
Schistocytes - helmet cells
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

14. What causes the jaundice in extravascular hemolysis
Nl - up - nl
Inc UCB
Signs and sx of aplastic anemia
A antigen and B antibodies

15. How are plasma cells characterized?
HIV or immunosupression
Valine for glutamate
Off center nuclues - clock face chromatin
HbSS

16. What do the labs show for TTP?
Dec plasma volume
Adult T cell lymphoma - presents with cutaneous lesions
Schistocytes and inc LDH
Inc lymphoctes - and less RS cells

17. Where to T cells arise and mature - and migrate to...
DIC
Def in factor IX
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Extravascular

18. What are dendritic cells called in the skin?
SLE - CLL - alpha methyldopa
Nl PT - elevated PTT - intrinsic pathway defect
Langerhans cells
All

19. Which immunoglobulin is involved in warm agglutination?
IgG - Warm is GREAT
Birbeck granules
No
Def in factor IX

20. What is the affected enzyme in acute intermittment porphyria
Valine for glutamate
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Porphobilinogen - delta ALA - uroporphyrin
VIII

21. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Valine for glutamate
Alpha thal - asian and african american
Bite cells and Heinz bodies
Extravascular

22. What is the mutation in HbS
Factor V resistant to activated protein C's inhibition
Hb Barts - gamma4 in defect in all 4 alpha genes
Common a few days after oxidative stress in a pt with G6PD
Valine for glutamate

23. What is the result of thrombocytopenia or platelet dysfunction?
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Inc
Schistocytes and inc LDH
Petechiae

24. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Off center nuclues - clock face chromatin
Schistocytes - helmet cells
Factor V Leiden
Spleen

25. What is the defect in beta thal?
No platelet clumping
Extravascular
Sideroblastic anemia
Splicing sites and promotor sequences

26. What is the life spance of a platelet?
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Fe def - thal - pb poison - sideroblastic anemia
Birbeck granules
8-10 days

27. Serum iron - transferrin - ferritin lab values for pregs - OCP use
IFN gama
Extrinsic - I - II - V - VII and X
Nl - up - nl
Beta 4

28. What is factor V leidin?

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29. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Signs and sx of aplastic anemia
DIC
Helminth infections major basic protein
Mantle cell - older males

30. What does the large SA:volume ratio in RBCs help facilitate?
SLE - CLL - alpha methyldopa
Easy gas exchange
Positive osmootic fragility test and splenectomy
IgG - Warm is GREAT

31. What is the ddx for aplastic anemia

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32. What are the neuro sx of B12 def?
B12/folate def
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Lung disease - congenital heart diseaes - and high altitude
120 days

33. What is the receptor for vWF
Uroporphyrinogen decarboxylase
Mantle cell - older males
Rhogam - Rh antigen immunoglobulin
Gp1b

34. Which factor does vWF carry/protect
B6
Fibrinogen bind GpIIb/IIIa and links platelts
Malaria - Babesia
VIII

35. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Mantle cell lymphoma
Protein S - cleaves and invactivates Va and VIIIa
Heinz bodies - seen in alpha thal and G6PD

36. When do you see MAHA?
No platelet clumping
Both alpha and beta thal
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Langerhans cell histiocytosis

37. What are the azuraphilic granules in PMNs
Contiguous
Lysosomes
Degrades fibrin mesh and converts C3 to C3a
Def in factor IX

38. What finding you do you see in patients after splenectomy
Howell Jolly bodies
Howell - Jolly bodies - hypo/asplenia
Hereditary spherocytosis
No platelet clumping

39. What is the pathogenesis of sickle cell
TXA2 - dec blood flow - inc platelet aggregation
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Hydroxyurea - bone marrow transplant
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

40. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
B12 def
Multiple myeloma
MGUS - monoclonal gammopathy of undetermined significance

41. What is the enzyme defect in Porphyria cutanea tarda
Porphyrias
Paroxysmal nocturnal hemoglobinuria
Uroporphyrinogen decarboxylase
DIC - TTP/HUS - traumatic hemolysis

42. Who are the professional APCs?
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Thrombotic thrombocytopenic purpura
Multiple myeloma
Dendritic cells?

43. Fava beans - sufla drugs - infectinos
Bleeding - malnutrition/absorption - inc demand pregs
Mantle cell lymphoma
Examples of oxidative stress
Ankryin - band 4.1 or spectrin

44. What is the tx for aplastic anemia
Folate def
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Varying sizes

45. Wilm's tumor - RCC - HCC - hydronephrosis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Inc serum iron - normal TIBC - inc ferratin
G6PD
MAHA

46. What is the tx for vWD
Contiguous
Aspirin
DDAVP (desmopressin) which releases stored vWF stored in endothelium
MAHA

47. What is monoclonal expansion without symptoms associated with multiple myeloma?
ATIII def
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Extravascular hemolysis
MGUS - monoclonal gammopathy of undetermined significance

48. Bone pain plus anemia
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Multiple myeloma
Glucose and heme - which inhibit ALA synthase
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

49. 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
Sickle cell
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Intrinsic - all factors except - VII - XIII
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

50. Bite cell
Her next Rh+ fetus
Porphobilinogen - delta ALA - uroporphyrin
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
G6PD