Immune thrombotic thrombocytopenic purpura in older patients: Results from the Spanish TTP Registry (REPTT).

Autor: Gómez-Seguí I; H. Universitario y Politécnico La Fe, Valencia, Spain., Francés Aracil E; H. Doctor Peset, Valencia, Spain., Mingot-Castellano ME; Hematology Department, Instituto de Biomedicina de Sevilla, H. U. Virgen del Rocio, Sevilla, Spain., Vara Pampliega M; H. Universitario Cruces, Barakaldo, Spain., Goterris Viciedo R; H. Clínico, Valencia, Spain., García Candel F; H. U. Virgen de la Arrixaca, Murcia, Spain., Pascual Izquierdo C; H. G. U. Gregorio Marañón, Madrid, Spain., Del Río Garma J; Complejo Hospitalario Universitario de Orense, Ourense, Spain., Guerra Domínguez L; Hospital Dr. Negrín, Las Palmas de Gran Canaria, Spain., Vicuña Andrés I; H. U. de La Princesa, Madrid, Spain., Garcia-Arroba Peinado J; H. Joan XXIII, Tarragona, Spain., Zalba Marcos S; H. Navarra, Pamplona, Spain., Vidan Estévez JM; H. de León, León, Spain., González Arias E; H. G. de Villalba, Madrid, Spain., Campuzano Saavedra V; H. U. de Burgos, Burgos, Spain., García Gala JM; H. Central de Asturias, Asturias, Spain., Ortega Sanchez S; Banc de Sang i Teixits, L'Hospitalet de Llobregat, Barcelona, Spain., Martínez Nieto J; Instituto de Investigación Biomédica del Hospital Clínico San Carlos, Madrid, Spain., Pardo Gambarte L; Fundación Jiménez Diaz, Madrid, Spain., Solé Rodríguez M; H. Juan Ramón Jiménez, Huelva, Spain., Fernández-Docampo M; H. Universitario A Coruña, A Coruña, Spain., Avila Idrovo LF; H. U. de Canarias, Canarias, Spain., Hernández L; H. G. U. de Alicante, Alicante, Spain., Cid J; H. Clínic, Barcelona, Spain., de la Rubia Comos J; H. Universitario y Politécnico La Fe, Valencia, Spain.; Hematology Department, Universidad Católica 'San Vicente Mártir', Valencia, Spain.
Jazyk: angličtina
Zdroj: British journal of haematology [Br J Haematol] 2023 Dec; Vol. 203 (5), pp. 860-871. Date of Electronic Publication: 2023 Sep 18.
DOI: 10.1111/bjh.19109
Abstrakt: Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.
(© 2023 British Society for Haematology and John Wiley & Sons Ltd.)
Databáze: MEDLINE