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. 2021 Aug;36(4):563-573.
doi: 10.1002/jca.21894. Epub 2021 Mar 29.

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

Cristina Pascual-Izquierdo  1   2 , Julio Del Rio-Garma  3 , Javier de la Rubia  4 , Aurora Viejo  5 , Eva Mingot  6 , Joan Cid  7 , Xavier Solanich  8 , Jesús Fernández-Sojo  9 , Jesús Martín-Sánchez  10 , Luis Hernández  11 , José María García-Gala  12 , Nieves Alonso  13 , Victoria González  14 , Ana Oliva  15 , Inés Gómez-Seguí  4 , Rosa Goterris  16 , Luisa Guerra  17 , Faustino García-Candel  18 , Marta Fernández-Docampo  19 , María Luisa Antelo  20 , Ángel Salgado-Barreira  21 , Ramón Salinas  22   23   24 ; Spanish Apheresis Group (GEA) and Spanish Thrombotic Thrombocytopenic Purpura Registry (REPTT)
Affiliations

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

Cristina Pascual-Izquierdo et al. J Clin Apher. 2021 Aug.

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.

Keywords: ADAMTS13; Spain; immune-mediated thrombotic thrombocytopenic purpura; incidence; treatment.

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References

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