TTP and atypical HUS share similar signs and symptoms. Although there is an overlap in clinical presentation, TTP is a disease of severe ADAMTS13 deficiency (≤10%), while levels of ADAMTS13 >10% in the context of microangiopathy should raise clinical suspicion of atypical HUS.
Consequences of TTP and atypical HUS make rapid diagnosis and clinical intervention critical; therefore, increased availability of a diagnostic test for ADAMTS13 activity is extremely important in delineating between TTP and atypical HUS. In an effort to achieve this goal, the following centres are now providing timely (24-48 hours) turnaround of ADAMTS13 testing. Note: blood draws for ADAMTS13 activity testing should be done prior to commencing plasma-based therapy.
Total: 7 results found.
Thrombotic microangiopathy (TMA) is a group of rare disorders affecting the microvasculture of most organ beds. The impact of these diseases on those affected by them is immense. Our understanding of these disorders is rapidly expanding and therapeutic options are beginning to appear. However, many challenges remain when managing TMA and one is a practical one: the diseases are rare and the experts in them are few and spread worldwide. Read more...