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- W2093846710 abstract "To the Editor.—Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder, classified as a thrombotic microangiopathy (TMA) based on clinical findings of hemolytic anemia, thrombocytopenia, neurologic symptoms, fever, and renal dysfunction.1 Unfortunately, several other TMAs have similar features (typical and atypical hemolytic uremic syndromes, disseminated intravascular coagulation, among others). An assay of ADAMTS13 is useful for distinguishing TTP from the other TMA disorders, thus identifying those patients who will benefit from plasma exchange (PE).2Barrows and Teruya3 reported their institution's experience using the ADAMTS13 assay, confirming that the test correctly identifies appropriate patients for PE. However, even with an in-house assay available, it may take hours to days to determine the ADAMTS13 result. Even that brief delay in making or refuting a diagnosis of TTP may be unacceptable to most physicians because urgent PE is the appropriate therapy.There are 2 solutions to this problem. One option is to subject all patients suspected of having TTP to PE until the ADAMTS13 result returns; however, that approach means that most patients who receive PE will be unnecessarily treated. More important, appropriate treatment for the underlying TMA disorder will be delayed. Another approach that we use at our institution is the establishment of clinical criteria for rapidly identifying patients who have a high probability, intermediate probability, or low probability of TTP.4 This clinical prediction score uses easily obtainable laboratory results (platelet count, d-dimer, creatinine, reticulocyte count, indirect bilirubin). Patients with a very low likelihood of TTP are not given PE, and alternative diagnoses are considered in those patients. Patients with an intermediate or a high likelihood of having TTP should receive PE. Results of this clinical prediction score were validated in a second cohort of patients with TMA.5We agree with Barrows and Teruya that ADAMTS13 testing is useful in diagnosing TTP. However, we disagree that “… the presence of thrombocytopenia and hemolytic anemia are generally sufficient for starting empiric treatment with TPE.”(p548) Patients with TTP represent only a few of the patients with TMA (5% in the authors' study, 10% in our studies), so exclusion of patients without TTP from receiving PE improves the efficiency of PE use and appropriate TMA management." @default.
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- W2093846710 date "2015-04-01" @default.
- W2093846710 modified "2023-10-18" @default.
- W2093846710 title "Utility of ADAMTS13 Assays in Diagnosing Thrombotic Thrombocytopenic Purpura" @default.
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- W2093846710 doi "https://doi.org/10.5858/arpa.2014-0225-le" @default.
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