TTP: thrombotic thrombocytopenic purpura; TPE: therapeutic plasma exchange; TMA: thrombotic microangiopathy; DIC: disseminated intravascular coagulation; MAHA: microangiopathic hemolytic anemia.
* Diagnosis is based on the finding of MAHA and thrombocytopenia without another explanation. Neurologic findings may be present but are not seen in all patients; kidney impairment is rare. We risk-stratify patients based on clinical criteria. ADAMTS13 activity testing should be performed as soon as possible, ideally before TPE is initiated; however, results may not be available for several days. Refer to UpToDate for details of the diagnostic evaluation, including the use of the PLASMIC score and other criteria that give high confidence in the diagnosis of TTP, which is based on clinical criteria rather than solely on ADAMTS13 activity.
¶ The following therapies are used in addition to TPE:Δ Response criteria are based on platelet count, with the exact details individualized according to the judgment of the treating clinician; refer to UpToDate for further discussion of these criteria.
◊ ADAMTS13 activity <10% is consistent with a diagnosis of TTP. Rarely, patients may have a slightly higher value; examples include an individual who has already undergone TPE before the blood sample was obtained. ADAMTS13 activity >20% is generally consistent with response to treatment or a condition other than TTP; in some cases, individuals with slightly lower values (10 to 20%) may have a condition other than TTP. If an individual has an increase in platelet count and ADAMTS13 activity >10 to 20%, therapy is individualized based on the clinical distinction between resolving TTP and another resolving cause of thrombocytopenia. Refer to UpToDate for further discussion of ADAMTS13 testing, test interference, and interpretation.
§ Refer to UpToDate for options if TTP does not respond to TPE, high-dose glucocorticoids, rituximab, and caplacizumab.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟