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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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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. How is beta thal minor dx
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
B symptoms - fever night sweats - weight loss
Inc HbA2 on electrophoresis
Tissue factor converst the VII and VIIa

2. What is the mutation in HbC
Howell Jolly bodies
Sideroblastic anemia
Lysine for glutamate at position 6
Petechiae

3. Ringed sideroblasts
Hodgkin
Sideroblastic anemia
Liver disease - abetalipoproteinemia - acntho = spiny
Splicing sites and promotor sequences

4. What is the treatment to prevent a woman from forming anti Rh antibody?
Easy gas exchange
Rhogam - Rh antigen immunoglobulin
Exposed collagen upon endothelial damage
Up - down - up

5. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Hereditary elloptocytosis
Splicing sites and promotor sequences
All trans retinoic acid

6. Where are 1/3 of platelets stored
Spleen
T(12;21)
Hereditary spherocytosis - G6PD - sickle cell
SLE - CLL - alpha methyldopa

7. Which drugs can cause macrocytic anemia?
EBV
5- FU - AZT - hydroxyurea
Off center nuclues - clock face chromatin
Examples of oxidative stress

8. Basophilica stippling
Leukemoid reaction
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Low in CML
Round densly staining nucleus with a small amount of pale cytoplasm

9. What do the platelets bind? What is the step called
G6PD
Hydroxyurea - bone marrow transplant
Tissue factor converst the VII and VIIa
They bind vWF via GpIb

10. What is the affected enzyme in lead poisoning
Tissue factor converst the VII and VIIa
Thrombotic thrombocytopenic purpura
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Ferrocheletase and ALA dehydrogenase

11. drug that inhibits ADP induced expression of GpIIb/IIIa
Sideroblastic anemia
Ticlopidine/clopidogrel
Prothrombin gene mutation
ADP and Ca

12. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Hereditary spherocytosis
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Leukemia
B6 therapy (pyrodixine)

13. Serum iron - transferrin - ferritin lab values for hemochromatosis
VIII
Up - down - up
Both alpha and beta thal
Blood

14. Target cell

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15. Why can newborns with sickle cell be asymptomatic
Inc HbF and dec HbS
MGUS - monoclonal gammopathy of undetermined significance
Glucose and heme - which inhibit ALA synthase
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

16. Serum iron - transferrin - ferritin lab values for iron def anemia
EBV
Hereditary elloptocytosis
Down - up - down
Inappropriate absolute with inc RBCs and EPO

17. Schisotcyte - helmet cell
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Low in CML
Langerhans cells
DIC - TTP/HUS - traumatic hemolysis

18. Plasma cell neoplasm
No platelet clumping
Multiple myeloma
Alpha thal - asian and african american
DIC

19. What do you see on peripheral smear with sideroblastic anemia
Inc RBC - dec O2 sat - inc EPO
Ringed sideronblasts with iron laden mitochondria
Inc lymphoctes - and less RS cells
Uroporphyrinogen decarboxylase

20. What is the ddx for aplastic anemia

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21. What are the age ranges for the various leukemias
Epoxide reductase - warfarin inhibits
Mycosis fundgoides/Sezary syndrome
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Bleeding - malnutrition/absorption - inc demand pregs

22. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
G6PD
Inactivates it
Deficiency in factor VIII
All

23. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
A antigen and B antibodies
Histaminase and arylsulfatase
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Sideroblastic anemia

24. What are the presenting symptoms of porphyria cutanea tarda
Epoxide reductase - warfarin inhibits
Eleveated PT - PTT
120 days
Blistering cutaneous photosens - most common porphyria

25. Megakaryocytosis
Essential thrombocytosis
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Glycine and succinyl - coa
Collagen - BM - activated platelets

26. What begins the extrinsic pathway?
Tissue factor converst the VII and VIIa
G6PD
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 -
B6

27. What are some causes of cold agglutinin anemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Malaria - Babesia
IFN gama

28. What is the characteristic histologic finding in Hodgkin Lymphoma
Down - up - down
Reed - sternberg cells
...
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

29. What is the presenting scenario for TTP?
Paroxysmal nocturnal hemoglobinuria
All trans retinoic acid
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Von Willebrand's disease

30. t(11:22)
Ewing sarcoma
Round densly staining nucleus with a small amount of pale cytoplasm
DIC
Hereditary spherocytosis - G6PD - sickle cell

31. What does the blood smear show in glanzmann's?
No platelet clumping
Complications of sickle cell anemia
Signs and sx of aplastic anemia
Lacunar in nodular sclerosing variant

32. What do the iron studies show in sideroblastic anemia
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Inc serum iron - normal TIBC - inc ferratin
Inc HbF and dec HbS
Intravascular hemolysis

33. What is the result of thrombocytopenia or platelet dysfunction?
Folate def
Plasma cell
Uroporphyrin (tea colored urine)
Petechiae

34. Drug that inhbits the GpIIb/IIIa directly
Langerhans cell histiocytosis
Abciximab
Hairy cell leukemia
Aplastic anemia - pancytopenia

35. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Lacunar in nodular sclerosing variant
Paroxysmal nocturnal hemoglobinuria
Acts to stabilize platelet plug
G6PD

36. What does antithrombin do and What activates it?
Sickle cell
Bite cells and Heinz bodies
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

37. What indicated a good prognosis in Hodgkin lymphoma?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Ewing sarcoma
Inc lymphoctes - and less RS cells
HIV or immunosupression

38. What is the receptor for fibrinogen?
M3 AML (acute promyelocytic leukemia)
GpIIb/IIIa
Ticlopidine/clopidogrel
Nl PT - elevated PTT - intrinsic pathway defect

39. What is the clinical picture of hemophilia A or B
VWF and fibrinogen
Extrinsic - I - II - V - VII and X
Parvovirus
Macrohemorrhage - hemarthroses - easy bruising

40. What is the pathogenesis of TTP?
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Inc large vWF multimers - inc platelet aggregation and thrombosis
Follicular lymphoma - indolent course
CML to AML or All

41. What is the characteristic finding for MAHA on peripheral smear?
Inc serum iron - normal TIBC - inc ferratin
B6
Schistocytes - helmet cells
Paraprotein spike - monoclonal protein

42. 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
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Anti Rh
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

43. What does ADP do?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
DIC - TTP/HUS - traumatic hemolysis
Helps platelts adhere to endothelium
Bone marrow - thymus - blood (80% of circulating lymphos are T)

44. What activates the intrinsic pathway?
No platelet clumping
Collagen - BM - activated platelets
B6
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

45. Deficiency in GpIIb/IIIa

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46. Basophilic nuclear remnants fonud in RBCs
Inc HbA2 on electrophoresis
Up - down - up
Howell - Jolly bodies - hypo/asplenia
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

47. Causes of iron def?
Collagen - BM - activated platelets
Bleeding - malnutrition/absorption - inc demand pregs
Malaria - Babesia
Porphobilinogen deaminase aka uroporphyrinogen I synthase

48. What are the likely exposures of kids and adults for lead poisoning
Megakaryocytes
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Fibrinogen
Kids= exposure to lead paint - adults = battery - ammunition factory

49. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Nodular sclerosing
Ewing sarcoma
All

50. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Yes - via MHC II
Signs and sx of aplastic anemia
Glycine and succinyl - coa
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination