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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 pathogenesis of sickle cell
Howell - Jolly bodies - hypo/asplenia
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Howell Jolly bodies
Idiopathic thrombocytopenia (ITP)
2. What is the Ddx for for a macrocytic anemia
Histamine - heparin - and eosinohil chemotactic factors
Rhogam - Rh antigen immunoglobulin
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
3. What substances are released in mast cell degranulation
Hodgkin
Histamine - heparin - and eosinohil chemotactic factors
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Hb Barts - gamma4 in defect in all 4 alpha genes
4. What are the neuro sx of B12 def?
Incactivates II - VII - IX - X - XI - XII
Antigen - antibody
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
5. What does NAACP stand for - in regards to the causes of eosinophiia?
...
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Protein C or S deficiency
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 -
6. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Histamine - heparin - and eosinohil chemotactic factors
Reed - sternberg cells
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
7. What is the effected enzyme in acute intermittment porphyria?
Gp1b
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Von Willebrand's disease
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
8. What is the coombs test results in AIHA?
Mantle cell - older males
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Pos
Hereditary spherocytosis - autoimmune hemolysis
9. Deficiency in GpIb
Beta chain underproduced - asymptomatic - heterozygote
Porphyrias
Bernard soulier
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 -
10. What is the activation stage of platelet plug formation?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
TRAP (tartrate resistant acid phosphatase
AB - no antibodies
11. What do platelets interact with to form a hemostatic plug
Dec plasma volume
Fibrinogen
Paraprotein spike - monoclonal protein
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
12. Drug that inhibits COX and therefore TXA2 synthesis
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Aspirin
Liver disease - abetalipoproteinemia - acntho = spiny
13. Schisotcyte - helmet cell
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Hereditary elloptocytosis
Lead - and EtOH
DIC - TTP/HUS - traumatic hemolysis
14. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
Sickle cell anemia
Hereditary spherocytosis - autoimmune hemolysis
Follicular lymphoma - indolent course
15. What does hairy cell leukemia stain with
G6PD
Extravascular
TRAP (tartrate resistant acid phosphatase
Inc RBC - dec O2 sat - inc EPO
16. Macro - ovalocyte
Lung disease - congenital heart diseaes - and high altitude
Protoporphyrin (blood)
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
ATIII def
17. Can B cells function as APCs?
M3 AML (acute promyelocytic leukemia)
Yes - via MHC II
Ankryin - band 4.1 or spectrin
Blasts > 5%
18. RBCs damagaed from passing through obstructed - narrowed vessel lumina
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
MAHA
Up - down - up
Hereditary spherocytosis - autoimmune hemolysis
19. What is the characteristic lab finding on electrophoresis
Histamine - heparin - and eosinohil chemotactic factors
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Paraprotein spike - monoclonal protein
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
20. How are the nucleus and the cytoplasm characterized for lymphocytes
Sickle cell anemia
AB - no antibodies
Blod - tissue - MACS
Round densly staining nucleus with a small amount of pale cytoplasm
21. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Inactivates it
DIC
22. What do labs show in ITP?
5- FU - AZT - hydroxyurea
Hereditary spherocytosis
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
23. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
HIV or immunosupression
IgG - Warm is GREAT
Idiopathic thrombocytopenia (ITP)
Lacunar in nodular sclerosing variant
24. What substance is Fe added to to yield heme
Low O2 in papilla; can also get microhematuria from medullary infarcts
Protoporphyrin
Malaria - Babesia
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 -
25. What does bradykinin do?
Schistocytes - helmet cells
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Inc vasodiltion - inc perm - inc pain
They bind vWF via GpIb
26. What does plasmin do?
Incactivates II - VII - IX - X - XI - XII
Blasts > 5%
Degrades fibrin mesh and converts C3 to C3a
TRAP (tartrate resistant acid phosphatase
27. What is the receptor for vWF
G6PD
Blasts > 5%
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Gp1b
28. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
PGI2 - NO inc blood flow - dec platelet aggregation
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
G6PD
29. What does the blood smear show in glanzmann's?
Leukemia
Essential thrombocytosis
No platelet clumping
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
30. Fibrotic obliteration of bone marow with teardrop cells
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31. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
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 -
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
CML
DIC
32. What is the purpose of the fibrin mesh
Low O2 in papilla; can also get microhematuria from medullary infarcts
Acts to stabilize platelet plug
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Heinz bodies - seen in alpha thal and G6PD
33. What is the pathogenesis of ACD?
Positive osmootic fragility test and splenectomy
CNS and testis
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Imatinib - anti bcr abl
34. inc LDH - jaundice
Cromolyn sodium
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Anti Rh
Extravascular hemolysis
35. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
36. What activates the fibrinolytic pathway?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Blod - tissue - MACS
Lead - and EtOH
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
37. Serum iron - transferrin - ferritin lab values for hemochromatosis
Ferrocheletase and ALA dehydrogenase
Both alpha and beta thal
Langerhans cell histiocytosis
Up - down - up
38. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
DIC - TTP/HUS - traumatic hemolysis
Blistering cutaneous photosens - most common porphyria
HbSS
Beta thal
39. What does decreasing heme do to ALA synthase activity?
20 to 40
Inc RBC - dec O2 sat - inc EPO
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Inc
40. What is the enzyme defect in Porphyria cutanea tarda
Complications of sickle cell anemia
Macrohemorrhage - hemarthroses - easy bruising
Uroporphyrinogen decarboxylase
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 -
41. What substance prevents mast cells degranulation?
Von Willebrand's disease
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Cromolyn sodium
SLE - CLL - alpha methyldopa
42. What is hemophiliia A
Essential thrombocytosis
Mantle cell lymphoma
Low in CML
Deficiency in factor VIII
43. Bone pain plus anemia
Collagen - BM - activated platelets
Multiple myeloma
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 -
IgG - Warm is GREAT
44. hemolytic in a newborn - dec ATP and rigid RBCs
Off center nuclues - clock face chromatin
Pyruvate kinase def - extravascular
CLL (SLL without the peripheral lymphocytosis
Hereditary spherocytosis - autoimmune hemolysis
45. deficiency in ADAMTS13 leading to dec degradation of vWF
Blasts > 5%
Both alpha and beta thal
Decrease EPO
Thrombotic thrombocytopenic purpura
46. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Leukemoid reaction
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Peroxidase
Heinz bodies - seen in alpha thal and G6PD
47. CD5+ - poor prognosis - t(11;14)
Aspirin
B6
Leukemia
Mantle cell - older males
48. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Positive osmootic fragility test and splenectomy
Induces differentiation of myeloblasts
49. What is the characteristic spread of Hodgkin Lymphoma
Nodular sclerosing
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Contiguous
50. What is the affected enzyme in acute intermittment porphyria
ADP and Ca
Orotic aciduria
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Porphobilinogen - delta ALA - uroporphyrin
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