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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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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 eosinophils defend against and What do they use to do it?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Sideroblastic anemia
Helminth infections major basic protein
Varying sizes

2. What activates protein C and What does activated protein C do?
Protein S - cleaves and invactivates Va and VIIIa
B symptoms - fever night sweats - weight loss
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Spleen

3. What are the variants of the RS cells
Off center nuclues - clock face chromatin
MAHA
Lacunar in nodular sclerosing variant
Epoxide reductase - warfarin inhibits

4. What percentage of WBCs are eosinophils?
1 to 6
CML
Extrinsic - I - II - V - VII and X
Down - down - up

5. Basophilica stippling
8-10 days
Inc HbF and dec HbS
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
40 to 75

6. What vitamin is a cofactor for the first step of heme synthesis
5- FU - AZT - hydroxyurea
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Birbeck granules
B6

7. What is hemophilia B?
DIC
EBV
Examples of oxidative stress
Def in factor IX

8. What signal activates MACS
IFN gama
Varying shapes
B12 def
CML to AML or All

9. What is the aggregation phase of ppf?
Fibrinogen bind GpIIb/IIIa and links platelts
Antigen - antibody
Mantle cell - older males
G6PD

10. adults - auer rods - inc circulating myeblasts on peripheral smear
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
B cells
TXA2 - dec blood flow - inc platelet aggregation
AML

11. What is the effected enzyme in acute intermittment porphyria?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Histaminase and arylsulfatase
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Porphobilinogen deaminase aka uroporphyrinogen I synthase

12. What do labs show in DIC?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Ticlopidine/clopidogrel
Complications of sickle cell anemia
Sideroblastic anemia

13. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
TPA - cleavage of fibrin mesh
Fc
B antigena and A antibodes

14. What causes the jaundice in extravascular hemolysis
Mantle cell lymphoma
Inc UCB
CML
Lung disease - congenital heart diseaes - and high altitude

15. iron deficiency anemia - esophageal web - atrophic glossitis
Protoporphyrin
No
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Plummer - vinson syndrome

16. Fava beans - sufla drugs - infectinos
Inc large vWF multimers - inc platelet aggregation and thrombosis
Ewing sarcoma
Examples of oxidative stress
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

17. Where are 1/3 of platelets stored
Lysosomes
Decrease EPO
Spleen
Bernard soulier

18. Elderly - mature b cell tumor with filamentous - hairlike projections
Low O2 in papilla; can also get microhematuria from medullary infarcts
Hairy cell leukemia
Africa = Jaw lesion - US = pelvis or abdomen
Her next Rh+ fetus

19. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Beta thal
Helminth infections major basic protein
VWD

20. 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
IgG - Warm is GREAT
Uroporphyrinogen decarboxylase
Protoporphyrin (blood)

21. What is a blast crisis
CML to AML or All
Anti Rh
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
AB - no antibodies

22. universal donor
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Exposed collagen upon endothelial damage
No antigen - both antibodies
DDAVP (desmopressin) which releases stored vWF stored in endothelium

23. What is the Ddx for for a macrocytic anemia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Dec plasma volume
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
No antigen - both antibodies

24. What does STOP Making New Thrombi stand for
Follicular lymphoma - indolent course
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

25. What is the pathogenesis of ACD?
Essential thrombocytosis
MAHA
Imatinib - anti bcr abl
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

26. Sickle cell
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Exposed collagen upon endothelial damage
Sickle cell anemia
Reed - sternberg cells

27. Conditions of defective heme synthesis leading to accumulation of heme precurors
Porphyrias
MGUS - monoclonal gammopathy of undetermined significance
Ringed sideronblasts with iron laden mitochondria
DDAVP (desmopressin) which releases stored vWF stored in endothelium

28. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Varying sizes
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

29. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
All
Diffuse Large b cell lymphoma
Multiple myeloma
DIC

30. What is the enzyme defect in Porphyria cutanea tarda
Multiple myeloma
T(12;21)
Uroporphyrinogen decarboxylase
Extravascular hemolysis

31. Which pathway and factors are tested in the PTT test
HIV or immunosupression
Intrinsic - all factors except - VII - XIII
Complications of sickle cell anemia
VWD

32. How does the therapy for M3 vairant work?
Induces differentiation of myeloblasts
Reed - sternberg cells
B antigena and A antibodes
Bone marrow - thymus - blood (80% of circulating lymphos are T)

33. What does antithrombin do and What activates it?
No antigen - both antibodies
Hydroxyurea - bone marrow transplant
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Porphyrias

34. t(11;14)
Def in factor IX
Mantle cell lymphoma
Bone marrow infiltration - myelofibrosis
CD15 and CD30 pos

35. What portion of IgE can mast cells bind
Fc
Paroxysmal nocturnal hemoglobinuria
Petechiae
Uroporphyrinogen decarboxylase

36. What is the Ddx for a normocytic - normochromic anemia?
Varying shapes
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Leukemoid reaction
No antigen - both antibodies

37. What does decreasing heme do to ALA synthase activity?
Protein C or S deficiency
Valine for glutamate
CML to AML or All
Inc

38. 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
Malaria - Babesia
Leukemia
Hydroxyurea - bone marrow transplant

39. What finding you do you see in patients after splenectomy
Myelofibrosis (marow is crying being its fibrosed'
Sideroblastic anemia
Howell Jolly bodies
Kids= exposure to lead paint - adults = battery - ammunition factory

40. What are the labs and tx for HS?
Both alpha and beta thal
Positive osmootic fragility test and splenectomy
Bite cells and Heinz bodies
Ewing sarcoma

41. What indicated a good prognosis in Hodgkin lymphoma?
120 days
Decrease EPO
Inc lymphoctes - and less RS cells
Adult T cell lymphoma - presents with cutaneous lesions

42. What is the tx for vWD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Hereditary spherocytosis - G6PD - sickle cell
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

43. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
Mycosis fundgoides/Sezary syndrome
MGUS - monoclonal gammopathy of undetermined significance
Inactivates it

44. What is the philadelphia chromosome
Eleveated PT - PTT
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Heinz bodies - seen in alpha thal and G6PD
T(9;22) bcr abl

45. What substance is Fe added to to yield heme
All
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Protoporphyrin

46. What is a metabolic disorder tht can cause macrocytic anemia?
Positive osmootic fragility test and splenectomy
Valine for glutamate
Orotic aciduria
SLE - CLL - alpha methyldopa

47. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
Lysine for glutamate at position 6
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Blood

48. What is the main source of energy in RBCs
Signs and sx of aplastic anemia
90% anearobically from glucose to lactate - 10% from HMP shunt
Induces differentiation of myeloblasts
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

49. What do you see on peripheral smear with sideroblastic anemia
Low O2 in papilla; can also get microhematuria from medullary infarcts
Blistering cutaneous photosens - most common porphyria
Ringed sideronblasts with iron laden mitochondria
Plummer - vinson syndrome

50. HTLV-1
IFN gama
Adult T cell lymphoma - presents with cutaneous lesions
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia