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Br J Haematol. 2015 Dec;171(5):836-44. doi: 10.1111/bjh.13658. Epub 2015 Aug 28.

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

Author information

1
Division of Hematology, Massachusetts General Hospital, Boston, MA, USA.
2
Harvard Medical School, Boston, MA, USA.
3
Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.
4
Division of Hematology and Oncology, Beth Israel Deaconess Medical Center, Boston, MA, USA.
5
Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA, USA.
6
Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.
7
Blood Transfusion Service, Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.

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 cohort that responds well to TPE. In contrast, TMA without severe ADAMTS13 deficiency is associated with increased mortality that may not be influenced by TPE.

KEYWORDS:

ADAMTS13; autoimmune thrombotic thrombocytopenic purpura; plasma exchange; thrombotic microangiopathy

PMID:
26314936
DOI:
10.1111/bjh.13658
[Indexed for MEDLINE]

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