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Hemeonc
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Subject
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health-sciences
Instructions:
Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.
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 is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
Plummer - vinson syndrome
A antigen and B antibodies
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
2. What are the presenting symptoms of acute intermittent porphyria
B antigena and A antibodes
Africa = Jaw lesion - US = pelvis or abdomen
A antigen and B antibodies
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
3. Conditions of defective heme synthesis leading to accumulation of heme precurors
CNS and testis
Porphyrias
Hb Barts - gamma4 in defect in all 4 alpha genes
Inactivates it
4. What does LEAD stand for in lead poisoning?
Hairy cell leukemia
Inc HbF and dec HbS
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 -
Direct coombs - agglutinate if RBCs are coated with Ig
5. What is the therapy for the M3 variant?
Indirect coombs - agglutinate if serum anti RBC surface Ig
They bind vWF via GpIb
MHC II and Fc receptors
All trans retinoic acid
6. HTLV-1
Reed - sternberg cells
No platelet clumping
Cromolyn sodium
Adult T cell lymphoma - presents with cutaneous lesions
7. What is the tx for sickle cell
AML
MGUS - monoclonal gammopathy of undetermined significance
Hydroxyurea - bone marrow transplant
Def in factor IX
8. What do auer rods stain with
Peroxidase
Exposed collagen upon endothelial damage
B6
Blasts > 5%
9. What is the Ddx for a normocytic - normochromic anemia?
Mycosis fundgoides/Sezary syndrome
Beta chain underproduced - asymptomatic - heterozygote
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Valine for glutamate
10. CD5+ - poor prognosis - t(11;14)
TXA2 - dec blood flow - inc platelet aggregation
Inc HbF and dec HbS
A antigen and B antibodies
Mantle cell - older males
11. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Both alpha and beta thal
VWD
Orotic aciduria
Howell Jolly bodies
12. What begins the extrinsic pathway?
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Rhogam - Rh antigen immunoglobulin
They bind vWF via GpIb
Tissue factor converst the VII and VIIa
13. What does decreasing heme do to ALA synthase activity?
Howell - Jolly bodies - hypo/asplenia
Inc
Glanzmann's throbmasthenia
No platelet clumping
14. t(8;14) c - myc gene
Burkitt lymphoma
Kids= exposure to lead paint - adults = battery - ammunition factory
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Liver disease - abetalipoproteinemia - acntho = spiny
15. What is the main source of energy in RBCs
Ticlopidine/clopidogrel
Parvovirus
90% anearobically from glucose to lactate - 10% from HMP shunt
Inc UCB
16. What are some causes of cold agglutinin anemia
Dendritic cells?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Anemia of chronic disease - Aplastic anemia - kidney disease
Inc serum iron - normal TIBC - inc ferratin
17. What is the general pathology of a macrocytic anemia?
40 to 50
Prothrombin gene mutation
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
18. Basophilic nuclear remnants fonud in RBCs
All trans retinoic acid
Howell - Jolly bodies - hypo/asplenia
TXA2 - dec blood flow - inc platelet aggregation
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
19. What is the characteristic lab finding on electrophoresis
Follicular lymphoma - indolent course
Paraprotein spike - monoclonal protein
90% anearobically from glucose to lactate - 10% from HMP shunt
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
20. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Hodgkin
Degrades fibrin mesh and converts C3 to C3a
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Alpha thal - asian and african american
21. What does ectopic EPO produce
Orotic aciduria
Fe def - thal - pb poison - sideroblastic anemia
Both alpha and beta thal
Inappropriate absolute with inc RBCs and EPO
22. What activates protein C and What does activated protein C do?
Extravascular hemolysis
Follicular lymphoma - indolent course
Von Willebrand's disease
Protein S - cleaves and invactivates Va and VIIIa
23. From what cells are platelets derived from
Megakaryocytes
Dendritic cells?
Paraprotein spike - monoclonal protein
2 to 10
24. After an injury - What does vWF bind to begin platelet plug formation?
Lung disease - congenital heart diseaes - and high altitude
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Exposed collagen upon endothelial damage
Prothrombin gene mutation
25. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
ATIII def
Gp1b
Both alpha and beta thal
Contiguous
26. What is the accumulated substance in lead poisoning
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
B6
Protoporphyrin (blood)
40 to 50
27. What do the platelets bind? What is the step called
Mantle cell lymphoma
TRAP (tartrate resistant acid phosphatase
They bind vWF via GpIb
Factor V Leiden
28. Megakaryocytosis
MAHA
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Essential thrombocytosis
G6PD
29. What converts plasminogen to plasm and What does plasmin do?
TPA - cleavage of fibrin mesh
Up - down - nl
Hereditary spherocytosis - G6PD - sickle cell
Langerhans cell histiocytosis
30. What is the difference of presentation of Burkitt in Africa vs the United States
Complications of sickle cell anemia
Lysosomes
Africa = Jaw lesion - US = pelvis or abdomen
Myelofibrosis (marow is crying being its fibrosed'
31. What are the main associations with multiple myeloma?
Multiple myeloma
Tissue factor converst the VII and VIIa
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
B12/folate def
32. t(11:22)
Nodular sclerosing
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Adult T cell lymphoma - presents with cutaneous lesions
Ewing sarcoma
33. universal donor
Fc
No antigen - both antibodies
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Porphobilinogen - delta ALA - uroporphyrin
34. 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
Sickle cell
Prothrombin gene mutation
Glanzmann's throbmasthenia
35. Deficiency in GpIb
Bernard soulier
Protein C or S deficiency
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Helps platelts adhere to endothelium
36. Bone pain plus anemia
IgG - Warm is GREAT
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Multiple myeloma
Bimodal - men>women - except for nodular sclerosing type
37. Acanthocyte (spur cell)
No platelet clumping
Liver disease - abetalipoproteinemia - acntho = spiny
Indirect coombs - agglutinate if serum anti RBC surface Ig
Degrades fibrin mesh and converts C3 to C3a
38. What is the pattern of involvement and spread for nonHod lympho
Schistocytes - helmet cells
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Plasma cell
Leukemia
39. What is appropriate absolute polycythemia
G6PD
B6
Inc RBC - dec O2 sat - inc EPO
Blood
40. Which pathway and factors are tested in the PTT test
Fc
Fe def - thal - pb poison - sideroblastic anemia
Intrinsic - all factors except - VII - XIII
Africa = Jaw lesion - US = pelvis or abdomen
41. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Bernard soulier
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Lead - and EtOH
Alpha thal - asian and african american
42. What do you see on peripheral smear with sideroblastic anemia
2 to 10
Polycythemia vera
Ringed sideronblasts with iron laden mitochondria
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
43. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Heinz bodies - seen in alpha thal and G6PD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
44. What are the anti aggregation factors?
Peroxidase
Plummer - vinson syndrome
Produce antibody - lots of RER and golgi
PGI2 - NO inc blood flow - dec platelet aggregation
45. Which cell is neoplastic in multiple myeloma
Uroporphyrin (tea colored urine)
Plasma cell
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Antigen - antibody
46. What causes hydrops fetalis
CML
Beta chain underproduced - asymptomatic - heterozygote
Direct coombs - agglutinate if RBCs are coated with Ig
Hb Barts - gamma4 in defect in all 4 alpha genes
47. Deficiency in vWF
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48. What percentage if WBCs are polys?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
40 to 75
Induces differentiation of myeloblasts
Reed - sternberg cells
49. What is the Ddx for nonhemolytic normocytic anemia
Inc HbF and dec HbS
Anemia of chronic disease - Aplastic anemia - kidney disease
Epoxide reductase - warfarin inhibits
No platelet clumping
50. What do platelets release necessary for coagulation cascaed?
ADP and Ca
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Lead - and EtOH
PGI2 - NO inc blood flow - dec platelet aggregation
Sorry!:) No result found.
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