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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 cell is primarily involved in non Hod lymph
Bite cells and Heinz bodies
B cells
Varying sizes
5- FU - AZT - hydroxyurea

2. What are the etiologies of folate def
EBV
Low in CML
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

3. What is the Ddx for a normocytic - normochromic anemia?
B symptoms - fever night sweats - weight loss
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
MAHA
Helminth infections major basic protein

4. What do platelets release necessary for coagulation cascaed?
Examples of oxidative stress
Heinz bodies - seen in alpha thal and G6PD
ADP and Ca
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

5. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Low O2 in papilla; can also get microhematuria from medullary infarcts
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

6. What vitamin is a cofactor for the first step of heme synthesis
Langerhans cells
B6
Sideroblastic anemia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

7. What do labs show in DIC?
Lysosomes
Blistering cutaneous photosens - most common porphyria
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Spleen

8. What is the life spance of a platelet?
All trans retinoic acid
ATIII def
8-10 days
B symptoms - fever night sweats - weight loss

9. What does ectopic EPO produce
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
SLE - CLL - alpha methyldopa
Inappropriate absolute with inc RBCs and EPO
Essential thrombocytosis

10. What does Vit K deficiency cause?
Macrohemorrhage - hemarthroses - easy bruising
Dec synthesis of factors 1972 - protein C/S
Bleeding - malnutrition/absorption - inc demand pregs
Beta thal

11. What is the general pathology of a macrocytic anemia?
Dec plasma volume
Schistocytes - helmet cells
B antigena and A antibodes
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

12. Bone pain plus anemia
Paraprotein spike - monoclonal protein
B6 therapy (pyrodixine)
Multiple myeloma
CML to AML or All

13. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
No lytic bone lesions in WM
Folate def
Histaminase and arylsulfatase
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

14. What is hemophilia B?
Aplastic anemia - pancytopenia
Down - up - down
Glucose and heme - which inhibit ALA synthase
Def in factor IX

15. What is the receptor for vWF
Inactivates it
Lysosomes
They bind vWF via GpIb
Gp1b

16. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Heinz bodies - seen in alpha thal and G6PD
VWF and fibrinogen
Low in CML
Essential thrombocytosis

17. What chromosomal translocation is associated with a better prognosis in All
G6PD
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
T(12;21)
Aplastic anemia - pancytopenia

18. deficiency in ADAMTS13 leading to dec degradation of vWF
CLL (SLL without the peripheral lymphocytosis
Thrombotic thrombocytopenic purpura
B antigena and A antibodes
GpIIb/IIIa

19. Is G6PD intravascular or extravascular
Peroxidase
Intravascular
CNS and testis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

20. What are dendritic cells called in the skin?
Beta chain underproduced - asymptomatic - heterozygote
Bite cells and Heinz bodies
Langerhans cells
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

21. What is makes a leukemia acute
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Yes - via MHC II
Direct coombs - agglutinate if RBCs are coated with Ig
Blasts > 5%

22. What substance accumulates in porphyria cutanea
Nodular sclerosing
Bleeding - malnutrition/absorption - inc demand pregs
Tissue
Uroporphyrin (tea colored urine)

23. t(8;14) c - myc gene
Burkitt lymphoma
Blistering cutaneous photosens - most common porphyria
TXA2 - dec blood flow - inc platelet aggregation
5- FU - AZT - hydroxyurea

24. What is the treatment for lead poising?
Paroxysmal nocturnal hemoglobinuria
Plasma cell
2 to 10
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

25. Ringed sideroblasts
Antigen - antibody
Peroxidase
Schistocytes and inc LDH
Sideroblastic anemia

26. Causes of iron def?
Mantle cell - older males
Aplastic anemia - pancytopenia
Bleeding - malnutrition/absorption - inc demand pregs
B cells

27. What is the tx for vWD
Follicular lymphoma - indolent course
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Protoporphyrin (blood)
Ewing sarcoma

28. How does vWD cause elevated PTT?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Bite cells and Heinz bodies
Fe def - thal - pb poison - sideroblastic anemia
VWF carries/protects factor VIII

29. What is the aggregation phase of ppf?
Glanzmann's throbmasthenia
Macrohemorrhage - hemarthroses - easy bruising
Fibrinogen bind GpIIb/IIIa and links platelts
Prothrombin gene mutation

30. What is the coombs test results in AIHA?
Pos
Inducers of primary antibody response
Inc HbF and dec HbS
Myelofibrosis (marow is crying being its fibrosed'

31. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Idiopathic thrombocytopenia (ITP)
Inc serum iron - normal TIBC - inc ferratin
DIC
Peroxidase

32. What is the enzyme defect in Porphyria cutanea tarda
Her next Rh+ fetus
Inc RBC - dec O2 sat - inc EPO
Uroporphyrinogen decarboxylase
No

33. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
All trans retinoic acid
A antigen and B antibodies
Hydroxyurea - bone marrow transplant

34. Which pathway and factorrs are tested by the PT coag test
Plasma cell
Extrinsic - I - II - V - VII and X
Leukemia
Birbeck granules

35. What is the difference of presentation of Burkitt in Africa vs the United States
Reed - sternberg cells
Orotic aciduria
Africa = Jaw lesion - US = pelvis or abdomen
No platelet clumping

36. What CD molecules are on RS cells
CD15 and CD30 pos
Lymphoma
Fe def - thal - pb poison - sideroblastic anemia
Myelofibrosis (marow is crying being its fibrosed'

37. What is the affected enzyme in lead poisoning
Imatinib - anti bcr abl
Lymphoma
Ferrocheletase and ALA dehydrogenase
Howell - Jolly bodies - hypo/asplenia

38. What does LEAD stand for in lead poisoning?
ATIII def
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 -
Lymphoma
Folate def

39. universal recipient
Bite cells and Heinz bodies
AB - no antibodies
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Inducers of primary antibody response

40. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
Ticlopidine/clopidogrel
Tissue factor converst the VII and VIIa
VWF and fibrinogen

41. What do eosinophils defend against and What do they use to do it?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Helminth infections major basic protein
Extravascular hemolysis
Bleeding - malnutrition/absorption - inc demand pregs

42. What do the platelets bind? What is the step called
VIII
Plummer - vinson syndrome
Multiple myeloma
They bind vWF via GpIb

43. Teardrop cell
Bone marrow infiltration - myelofibrosis
Glycine and succinyl - coa
ATIII def
Inc serum iron - normal TIBC - inc ferratin

44. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Blasts > 5%
Off center nuclues - clock face chromatin
Lead - and EtOH

45. What activates the fibrinolytic pathway?
Up - down - up
G6PD
ADP and Ca
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

46. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Leukemia
Beta chain underproduced - asymptomatic - heterozygote
Extrinsic - I - II - V - VII and X
Inc large vWF multimers - inc platelet aggregation and thrombosis

47. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
Schistocytes and inc LDH
Sideroblastic anemia
Porphobilinogen deaminase aka uroporphyrinogen I synthase

48. What are the pro aggregation factors?
Plasma cell
Helps platelts adhere to endothelium
TXA2 - dec blood flow - inc platelet aggregation
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

49. What is the clinical picture of hemophilia A or B
Exposed collagen upon endothelial damage
Macrohemorrhage - hemarthroses - easy bruising
TPA - cleavage of fibrin mesh
Plasma cell

50. What portion of IgE can mast cells bind
Fc
Africa = Jaw lesion - US = pelvis or abdomen
Aplastic anemia - pancytopenia
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA