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When Diagnosing Thrombotic Thrombocytopenic Purpura, Speed Is Critical
Thrombotic thrombocytopenic purpura (TTP) requires empirical therapy with plasma exchange, steroids, and occasionally the nanobody caplacizumab (if the hematologist's clinical suspicion is intermediate or high). Therapy is continued if activity of ADAMTS13 confirms the diagnosis or discontinued when testing rules out the condition. Because testing turnaround at most institutions can take several days, commercial assays with rapid turnaround time (i.e., <1 hour) have been developed. To evaluate the performance of three such tests, investigators conducted a systematic review and meta-analysis (19 studies, 4207 test samples) that compared rapid assays to reference standards.
All three rapid assays demonstrated high sensitivity and negative predictive value, but one was favored due to its optimal performance — the HemosIL AcuStar chemiluminescence immunoassay had sensitivity of 98% and negative predicted value of 99%.
Comment
I agree with the authors that TTP is a “can't-miss” diagnosis, and one of the hematologic emergencies that residents and fellows are taught early on to suspect. At my institution, ADAMTS13 activity (with reflex to inhibitor) is sent out to a reference lab, adding several days to the turnaround time. Thus, I'd value a rapid assay that could forestall the need for empirical therapy and eliminate the possibility of complications such as reactions to plasma, vascular access thrombosis or infection, and adverse events associated with caplacizumab itself. Moreover, this quick, low-cost approach is relatively easy to adopt, even in smaller community settings — and would focus the differential diagnosis on other “can't-miss” microangiopathic hemolytic anemia syndromes.
Citation(s)
Author:
Deshpande SR et al.
Title:
Rapid ADAMTS13 activity assays for thrombotic thrombocytopenic purpura: A systematic review and meta-analysis.
Source:
Blood
2025
Jul
10; [e-pub].
(Abstract/FREE Full Text)
Empfohlen von
Brady L. Stein, MD, MHS