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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. anti - Ig antibody added to patients RBCs;
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Blood
Kids= exposure to lead paint - adults = battery - ammunition factory
Direct coombs - agglutinate if RBCs are coated with Ig

2. What reveresible things can a sideroblastic anemia
Inc UCB
Lung disease - congenital heart diseaes - and high altitude
Uroporphyrin (tea colored urine)
Lead - and EtOH

3. What does plasmin do?
ATIII def
Degrades fibrin mesh and converts C3 to C3a
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Prothrombin gene mutation

4. What is the most common nonhodgkin lymphoma
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Diffuse Large b cell lymphoma
Plasma cell
M3 AML (acute promyelocytic leukemia)

5. What condition can result from treating AML M3 from the release of the Auer rods
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
DIC
VWF carries/protects factor VIII
SLE - CLL - alpha methyldopa

6. What is the pathogenesis of TTP?
HIV or immunosupression
Bernard soulier
Lead - and EtOH
Inc large vWF multimers - inc platelet aggregation and thrombosis

7. What do you see in peripheral smear in a pt with G6PD?
Direct coombs - agglutinate if RBCs are coated with Ig
Ankryin - band 4.1 or spectrin
Bite cells and Heinz bodies
EBV

8. Bite cell
Signs and sx of aplastic anemia
Inc HbA2 on electrophoresis
Hereditary spherocytosis - G6PD - sickle cell
G6PD

9. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Macrohemorrhage - hemarthroses - easy bruising
ATIII def
Direct coombs - agglutinate if RBCs are coated with Ig

10. What are the four levels of alpha thal?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Degrades fibrin mesh and converts C3 to C3a
Splicing sites and promotor sequences

11. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Up - down - nl
Positive osmootic fragility test and splenectomy
Idiopathic thrombocytopenia (ITP)

12. What do you see on peripheral smear with sideroblastic anemia
Blasts > 5%
Beta thal
Def in factor IX
Ringed sideronblasts with iron laden mitochondria

13. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
No lytic bone lesions in WM
Langerhans cell histiocytosis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

14. What is the age group most commonly affected by multiple myeloma
40 to 50
Hereditary spherocytosis - autoimmune hemolysis
VWD
Hereditary spherocytosis - G6PD - sickle cell

15. Serum iron - transferrin - ferritin lab values for hemochromatosis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Eleveated PT - PTT
Up - down - up
Mantle cell lymphoma

16. What is the characteristic histologic finding in Hodgkin Lymphoma
Protein S - cleaves and invactivates Va and VIIIa
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Reed - sternberg cells
Bleeding - malnutrition/absorption - inc demand pregs

17. What is monoclonal expansion without symptoms associated with multiple myeloma?
MGUS - monoclonal gammopathy of undetermined significance
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Lacunar in nodular sclerosing variant
Leukemia

18. What is the life span of a normal RBC
120 days
CML to AML or All
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
VWD

19. Where are mast cells found
Tissue
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Def in factor IX

20. mother's antibodies attack fetal 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 -
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
DIC - TTP/HUS - traumatic hemolysis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

21. inc LDH - jaundice
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Leukemia
Lysosomes
Extravascular hemolysis

22. Why does B12 def cause neuro sx?
Positive osmootic fragility test and splenectomy
Ringed sideronblasts with iron laden mitochondria
B12 in fatty acid pathways leads to subacute combined degeneration
Von Willebrand's disease

23. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Inappropriate absolute with inc RBCs and EPO
2 to 10
Lung disease - congenital heart diseaes - and high altitude

24. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Beta thal
Complications of sickle cell anemia
Tissue
ADP and Ca

25. S-100 and CD1a with birbeck granules
M3 AML (acute promyelocytic leukemia)
Langerhans cell histiocytosis
All
HIV or immunosupression

26. t(8;14) c - myc gene
Burkitt lymphoma
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Deficiency in factor VIII
Ringed sideronblasts with iron laden mitochondria

27. What virus is associated with Burkitt lymphoma
Birbeck granules
Africa = Jaw lesion - US = pelvis or abdomen
Nl PT - elevated PTT - intrinsic pathway defect
EBV

28. What do eosinophils defend against and What do they use to do it?
Helminth infections major basic protein
AB - no antibodies
Common a few days after oxidative stress in a pt with G6PD
Inc

29. What is HbH
40 to 75
Beta 4
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Gp1b

30. Where are 1/3 of platelets stored
Paroxysmal nocturnal hemoglobinuria
Spleen
Acts to stabilize platelet plug
Pos

31. What finding you do you see in patients after splenectomy
Inc RBC - dec O2 sat - inc EPO
Howell Jolly bodies
Idiopathic thrombocytopenia (ITP)
ADP and Ca

32. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Leukemia
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Uroporphyrinogen decarboxylase
Nl - up - nl

33. What is the receptor for fibrinogen?
Decrease EPO
Paraprotein spike - monoclonal protein
GpIIb/IIIa
Hereditary spherocytosis

34. What is the treatment for lead poising?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Easy gas exchange
MHC II and Fc receptors
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

35. What is the receptor for vWF
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inc RBC - dec O2 sat - inc EPO
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Gp1b

36. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Plasma cell
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
All

37. iron deficiency anemia - esophageal web - atrophic glossitis
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Epoxide reductase - warfarin inhibits
Varying shapes
Plummer - vinson syndrome

38. Bone pain plus anemia
Histamine - heparin - and eosinohil chemotactic factors
Multiple myeloma
Megakaryocytes
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

39. Back pain - hemoglobinuria
MHC II and Fc receptors
Myelofibrosis (marow is crying being its fibrosed'
Common a few days after oxidative stress in a pt with G6PD
Folate def

40. Elliptocyte
Hereditary elloptocytosis
Positive osmootic fragility test and splenectomy
Pos
Extrinsic - I - II - V - VII and X

41. universal donor
No antigen - both antibodies
Produce antibody - lots of RER and golgi
Von Willebrand's disease
MAHA

42. Which substrates begin the heme synthesis pathway
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
Nodular sclerosing

43. What is the life spance of a platelet?
Fibrinogen
Diffuse Large b cell lymphoma
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
8-10 days

44. What are the variants of the RS cells
TXA2 - dec blood flow - inc platelet aggregation
B12 def
Nodular sclerosing
Lacunar in nodular sclerosing variant

45. What is the difference of presentation of Burkitt in Africa vs the United States
B cells
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Africa = Jaw lesion - US = pelvis or abdomen
Anti Rh

46. What is the ddx for aplastic anemia

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47. What are the extrinsic hemolytic normocytic anemias?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Common a few days after oxidative stress in a pt with G6PD
Helminth infections major basic protein
...

48. What is the characteristic finding for MAHA on peripheral smear?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Schistocytes - helmet cells
Aspirin
Bimodal - men>women - except for nodular sclerosing type

49. What substance prevents mast cells degranulation?
Cromolyn sodium
AML
Varying sizes
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

50. Who has more severe disease - HbSS or HbSC
Megakaryocytes
Down - down - up
HbSS
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)