EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS

Objective: Acquired thrombotic thrombocytopenic purpura (aTTP) is an ultra-rare, potentially life-threatening thrombotic microangiopathy (TMA). Data on epidemiology, disease management, and clinical outcomes are scarce and often heterogeneous. The aim of this study was to assess the epidemiology, di...

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Autores principales:	Ayoade Adeyemi, Filip Callewaert, Rui de Passos Sousa, Francesca Razakariasa
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/6f7ff9af54a04e6e8f56b6762bf941b8
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spelling	oai:doaj.org-article:6f7ff9af54a04e6e8f56b6762bf941b82021-11-10T04:36:46ZEPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS2531-137910.1016/j.htct.2021.10.1048https://doaj.org/article/6f7ff9af54a04e6e8f56b6762bf941b82021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2531137921011950https://doaj.org/toc/2531-1379Objective: Acquired thrombotic thrombocytopenic purpura (aTTP) is an ultra-rare, potentially life-threatening thrombotic microangiopathy (TMA). Data on epidemiology, disease management, and clinical outcomes are scarce and often heterogeneous. The aim of this study was to assess the epidemiology, disease management, and clinical outcomes in patients with aTTP in the United States. Methodology: This longitudinal retrospective observational study of the Optum-Humedica database included patients with aTTP diagnosis from October 2015 to December 2019 if they had ≥1 documented ADAMTS13 activity <10% or ≥1 aTTP episode (≥1 inpatient stay with TMA diagnosis and ≥1 therapeutic plasma exchange [TPE] during the same stay); patients with conditions that mimic aTTP were excluded. Patients were followed until loss to follow-up, end of study period, or death. All analyses were descriptive. Results: Among 666 patients with aTTP diagnosis, 302 (45%) had ≥1 aTTP episode. Annual incidence of ≥1 aTTP episode was 1.81/million (based on data from 2016–2019). Patients with ≥1 aTTP episode received a mean of 16.7 TPE sessions; 59% used rituximab. Among patients with ≥1 aTTP episode, exacerbations occurred in 17% (52/302); relapse occurred in 11% (34/302). Mortality rate was 25% (167/666) among all patients with aTTP diagnosis and 14% (41/302) among patients with ≥1 aTTP episode. Conclusion: Despite treatment with TPE and immunosuppressants, the high mortality and morbidity observed in this patient population demonstrates the need for more effective therapies to improve clinical outcomes.Ayoade AdeyemiFilip CallewaertRui de Passos SousaFrancesca RazakariasaElsevierarticleDiseases of the blood and blood-forming organsRC633-647.5ENHematology, Transfusion and Cell Therapy, Vol 43, Iss , Pp S47- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Diseases of the blood and blood-forming organs RC633-647.5 Ayoade Adeyemi Filip Callewaert Rui de Passos Sousa Francesca Razakariasa EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
description	Objective: Acquired thrombotic thrombocytopenic purpura (aTTP) is an ultra-rare, potentially life-threatening thrombotic microangiopathy (TMA). Data on epidemiology, disease management, and clinical outcomes are scarce and often heterogeneous. The aim of this study was to assess the epidemiology, disease management, and clinical outcomes in patients with aTTP in the United States. Methodology: This longitudinal retrospective observational study of the Optum-Humedica database included patients with aTTP diagnosis from October 2015 to December 2019 if they had ≥1 documented ADAMTS13 activity <10% or ≥1 aTTP episode (≥1 inpatient stay with TMA diagnosis and ≥1 therapeutic plasma exchange [TPE] during the same stay); patients with conditions that mimic aTTP were excluded. Patients were followed until loss to follow-up, end of study period, or death. All analyses were descriptive. Results: Among 666 patients with aTTP diagnosis, 302 (45%) had ≥1 aTTP episode. Annual incidence of ≥1 aTTP episode was 1.81/million (based on data from 2016–2019). Patients with ≥1 aTTP episode received a mean of 16.7 TPE sessions; 59% used rituximab. Among patients with ≥1 aTTP episode, exacerbations occurred in 17% (52/302); relapse occurred in 11% (34/302). Mortality rate was 25% (167/666) among all patients with aTTP diagnosis and 14% (41/302) among patients with ≥1 aTTP episode. Conclusion: Despite treatment with TPE and immunosuppressants, the high mortality and morbidity observed in this patient population demonstrates the need for more effective therapies to improve clinical outcomes.
format	article
author	Ayoade Adeyemi Filip Callewaert Rui de Passos Sousa Francesca Razakariasa
author_facet	Ayoade Adeyemi Filip Callewaert Rui de Passos Sousa Francesca Razakariasa
author_sort	Ayoade Adeyemi
title	EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
title_short	EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
title_full	EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
title_fullStr	EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
title_full_unstemmed	EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS
title_sort	epidemiology, treatment patterns, and clinical outcomes among patients with acquired thrombotic thrombocytopenic purpura (attp) in the united states: an electronic health records analysis
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/6f7ff9af54a04e6e8f56b6762bf941b8
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_version_	1718440567358095361

EPIDEMIOLOGY, TREATMENT PATTERNS, AND CLINICAL OUTCOMES AMONG PATIENTS WITH ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IN THE UNITED STATES: AN ELECTRONIC HEALTH RECORDS ANALYSIS

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