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. 2025 Jun 1;110(6):1368-1372.
doi: 10.3324/haematol.2024.285391. Epub 2024 Sep 26.

Acquired thrombotic thrombocytopenic purpura without detectable anti-ADAMTS13 antibodies: a possible underlying autoimmune mechanism

Delphine Simon  1 , Mathilde Leclercq  2 , Bérangère Joly  3 , Agnès Veyradier  3 , Paul Coppo  4 , Ygal Benhamou  5
Affiliations

Acquired thrombotic thrombocytopenic purpura without detectable anti-ADAMTS13 antibodies: a possible underlying autoimmune mechanism

Delphine Simon et al. Haematologica. .

Abstract

In up to 25% of patients with acquired TTP, anti-ADAMTS13 antibodies are not identified, the mechanism resulting from ADAMTS13 deficiency remains unidentified (uTTP). In this study, we provide further insights on clinical presentation and outcome of uTTP. In patients with baseline undetectable anti-ADAMTS13 antibodies, usual features of iTTP (young age, cerebral involvement, severe thrombocytopenia) with no other associated context than a history of systemic autoimmune disease or pregnancy, should prompt to consider the diagnosis of iTTP.

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Figures

Figure 1.
Figure 1.
Flow-chart of the study population. TMA: thrombotic microangiopathy; ADAMTS13: a disintegrin and metalloprotease with thrombospondin type-1 repeats, member 13; TTP: thrombotic thrombocytopenic purpura; iTTP: immune-mediated thrombotic thrombocytopenic purpura; uTTP: thrombotic thrombocytopenic purpura of unidentified mechanism.
See this image and copyright information in PMC

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