Acquired thrombotic thrombocytopenic purpura without detectable anti-ADAMTS13 antibodies: a possible underlying autoimmune mechanism.
Delphine Simon,
Mathilde Leclercq,
Bérangère Joly,
Agnès Veyradier,
Paul Coppo,
Ygal Benhamou
Affiliations
Delphine Simon
Département de Médecine interne, CHU Charles Nicolle, F-76000 Rouen
Mathilde Leclercq
Département de Médecine interne, CHU Charles Nicolle, F-76000 Rouen, France; Université de Rouen, INSERM U1234 PANTHER, F-76000 Rouen
Bérangère Joly
Département d’Hématologie biologique, Hôpital Lariboisière, AP-HP, F-75000, Paris, France; Equipe d’accueil 3518, Institut de Recherche Saint-Louis, Université de Paris, F-75000, Paris
Agnès Veyradier
Département d’Hématologie biologique, Hôpital Lariboisière, AP-HP, F-75000, Paris, France; Equipe d’accueil 3518, Institut de Recherche Saint-Louis, Université de Paris, F-75000, Paris
Paul Coppo
Centre de Référence des Microangiopathies Thrombotiques, Service d’Hématologie, APHP et Sorbonne Université, F-75000, Paris, France; INSERM Unité Mixte de Recherche (UMRS) 1138, Centre de Recherche des Cordeliers, F-75000, Paris
Ygal Benhamou
Département de Médecine interne, CHU Charles Nicolle, F-76000 Rouen, France; Université de Rouen, INSERM U1026 EnVI, F-76000 Rouen
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.
