Incidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura

Fecha de publicación: 01/08/2021 Fecha Ahead of Print: 01/03/2021

Autores de IIS La Fe

Javier De La Rubia Comos

Autor
Inés Gómez Seguí

Autor

Participantes ajenos a IIS La Fe

Pascual-Izquierdo, C
del Rio-Garma, J
Viejo, A
Mingot, E
Cid, J
Solanich, X
Fernandez-Sojo, J
Martin-Sanchez, J
Hernandez, L
Garcia-Gala, JM
Alonso, N
Gonzalez, V
Oliva, A
Goterris, R
Guerra, L
Garcia-Candel, F
Fernandez-Docampo, M
Antelo, ML
Salgado-Barreira, A
Salinas, R

Grupos

Hematología y Hemoterapia

Investigador Principal

Javier De La Rubia Comos

Abstract

Background Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare disease characterized by the presence of anti-ADAMTS13 autoantibodies. Achieving accurate information on incidence and customary disease management is important to provide appropriate diagnostic and therapeutic resources. The aim of this study was to determine the incidence and outcomes of iTTP in Spain. Study design and methods A cross-sectional survey was carried out among Spanish hospitals, focused on iTTP patients >= 16 years old attended between 2015 and 2017, and those at follow-up before that interval. Incidence, prevalence, mortality, refractoriness, exacerbations, treatment complications, relapses, and sequelae were estimated. Results Forty-two hospitals covering roughly 20 million inhabitants answered the survey and reported 203 episodes (138 newly-diagnosed and 65 relapses), of which 193 (95.1%) were treated. Incidence was 2.67 (95% CI 1.90-3.45) patients per million inhabitants per year and prevalence 21.44 (95% CI% 19.10-23.73) patients per million inhabitants. At diagnosis, ADAMTS13 activity and anti-ADAMTS13 autoantibody were measured in 97% and 84.3% of reported episodes, respectively. Fifteen patients (7.4%) died as a direct consequence of iTTP, 6 of them before receiving any iTTP-specific treatment. Thirty-one (16.1%) of the 193 treated episodes were refractory to plasma exchange and corticosteroids, and 51 (26.4%) suffered at least one exacerbation. Conclusion iTTP incidence and prevalence were somewhat higher than those documented in neighboring countries. Together with data on treatments and outcomes, this information will allow us to better estimate what is needed to improve diagnosis and prognosis of iTTP patients in Spain.

Datos de la publicación

ISSN/ISSNe:: 0733-2459, 1098-1101
Tipo:: Article
Páginas:: 563-573
DOI:: 10.1002/jca.21894
Factor de Impacto:: 0,591 SCImago ℠
Cuartil:: Q2 SCImago ℠

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Keywords

ADAMTS13; immune‐ mediated thrombotic thrombocytopenic purpura; incidence; Spain; treatment

Campos de estudio

Proyectos asociados

RED DE INVESTIGACION COOPERATIVA DE CANCER

RD06/0020/0031 . INSTITUTO DE SALUD CARLOS III; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2006

CREACION Y MEJORA DE INFRAESTRUCTURAS EN MEDICINA REGENERATIVA

Investigador Principal: FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA

PLE2009-0141 ACI-PLAN E . MINISTERIO DE CIENCIA E INNOVACION; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2009