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13 Cards in this Set
- Front
- Back
What is TTP etiology?
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Sporadic caases are due to an antibody against the protease which cleaves the ultra-large molecular wt multimers of von Willebrand's factor.
The protease is called ADAMTS-13, which stands for A Disintegrin And Metalloprotease with Thrombospondin like repeats... more |
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treatment for TTP?
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plasma exchange
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should platelet transfusion be used for ttp tx?
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no- they fuel the fire
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what are the unique features of TTP/HUS?
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fever, neuro sequelae, but more renal manifestiations. usually precipitated by dirrheal illness. esp e.coli 0157:H7 or shigella . more in ped pts. respond less well to plasma exchange
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what drugs are clinically induce thromocytomenia?
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B-lactam antibiotics, Trimethoprim-sulfamethoxazole and other sulfa drugs, quinine/quinidine, heparin, abciximab (ReoPro)
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what is heparin induced thrombocytopenia?
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1-3% of pts with heparin get it 7-10 days after heparin, platelts fall by 30-50%. plattlet counts do not have to be normal. if platelets fall on heparin, stop heparin immediately. can lead, paradoxically to thrombosis in some pts.
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ITP?
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need to exclude splenomegaly and subclinical thyroid disease. megakaryocytes should be present inthe marrow because it is a peripheral descructive process. in adults when platelt count < 50, get tx.
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ITP in kids?
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in kids is is usually provoked by viral illness and no tx needed.
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itp tx?
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corticosteriods, take 48-72 hrs to take effect.
if platelet count <10K, or if pt bleeding, need more rapid Tx, use IVIg if pt is Rh +, cuse Anti-D in place of IVIg, acts as a decoy for splenic macrophages |
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ITP Therapy?
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2/3 of adult pts will relapse after steriod taper. in this case, splenectomy is required. 2/3 of pts respond to splenectomy. otherwise, more immunosuppresson
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splenic sequestration?
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normally 1/3 of total platelet mass resides in spleen. splenic enlargement from any cause can lead to displacement of platelets from peripheral circulaton into splenic pool.
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splenimegaly causes?
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Portal Hypertension
Malignancy Chronic Hemolytic Diseases-Hemoglobin C (not Sickle Cell Disease, they have small spleen!), Thallassemias, Hereditary Sperocytosis. |
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Thrombocytosis?
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too many platelets!
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