Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative

British Journal of Haematology
Pavan K BendapudiRobert S Makar

Abstract

The Harvard TMA Research Collaborative is a multi-institutional registry-based effort to study thrombotic microangiopathies (TMA). Laboratory and clinical parameters were recorded for 254 cases of suspected autoimmune thrombotic thrombocytopenic purpura (TTP). Patients with severe ADAMTS13 deficiency (activity ≤10%, N = 68) were more likely to be young, female and without a history of cancer treatment or transplantation. While all patients with severe deficiency were diagnosed with autoimmune TTP, those without severe deficiency frequently had disseminated intravascular coagulation, drug-associated TMA and transplant-related TMA. Patients with severe ADAMTS13 deficiency had superior overall survival at 360 d compared to those without severe deficiency (93·0% vs. 47·5%, P < 0·0001). Almost all patients with severe deficiency received therapeutic plasma exchange (TPE), but the use of TPE in patients with ADAMTS13 activity >10% varied significantly across the institutions in our consortium (13·2-63·8%, P < 0·0001). Nevertheless, 90-d mortality was not different in patients with ADAMTS13 activity >10% between the three hospitals (P = 0·98). Our data show that patients with severe ADAMTS13 deficiency represent a clinically distinct ...Continue Reading

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Citations

Feb 13, 2016·American Journal of Clinical Pathology·Pavan K Bendapudi, Robert S Makar
Jul 6, 2016·Journal of Thrombosis and Thrombolysis·Giulia Berti de MarinisAnna Maria Lombardi
Sep 20, 2018·Revista Do Instituto De Medicina Tropical De São Paulo·Maria Luiza Almeida BastosGeraldo Bezerra da Silva Junior
Dec 7, 2018·Hematology·Thita Chiasakul, Adam Cuker
Apr 20, 2020·Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy·Rui-Xue SunJing Yang
Jul 17, 2020·International Journal of Laboratory Hematology·Ian MackieRoss Baker
Sep 12, 2020·Journal of Thrombosis and Haemostasis : JTH·X Long ZhengFlora Peyvandi
Apr 28, 2017·International Journal of Laboratory Hematology·D S Chabot-Richards, K Foucar
Apr 4, 2020·Expert Review of Hematology·Andrés Gómez-De LeónDavid Gómez-Almaguer
Mar 20, 2021·Frontiers in Medicine·Nicola OstiSimonetta Friso
May 12, 2021·Journal of Thrombosis and Haemostasis : JTH·Jeremy SchofieldTina Dutt
Jul 20, 2021·Expert Review of Hematology·Inés Gómez-SeguíJavier de la Rubia Comos
Oct 14, 2021·Annals of Hematology·Julio Del Río-GarmaUNKNOWN “Registro Español de la Púrpura Trombocitopénica Trombótica (REPTT)”, and the “Grupo Español de Aféresis (GEA)”

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