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13 Cards in this Set

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What is TTP etiology?
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
treatment for TTP?
plasma exchange
should platelet transfusion be used for ttp tx?
no- they fuel the fire
what are the unique features of TTP/HUS?
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
what drugs are clinically induce thromocytomenia?
B-lactam antibiotics, Trimethoprim-sulfamethoxazole and other sulfa drugs, quinine/quinidine, heparin, abciximab (ReoPro)
what is heparin induced thrombocytopenia?
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.
ITP?
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.
ITP in kids?
in kids is is usually provoked by viral illness and no tx needed.
itp tx?
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
ITP Therapy?
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
splenic sequestration?
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.
splenimegaly causes?
Portal Hypertension
Malignancy
Chronic Hemolytic Diseases-Hemoglobin C (not Sickle Cell Disease, they have small spleen!), Thallassemias, Hereditary Sperocytosis.
Thrombocytosis?
too many platelets!