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

Autores de IIS La Fe

• Maria Eva Mingot Castellano

  Autor

• 

  Javier De La Rubia Comos

  Autor

Participantes ajenos a IIS La Fe

• Gomez-Segui, I
• Frances Aracil, E
• Vara Pampliega, M
• Goterris Viciedo, R
• Garcia Candel, F
• Pascual Izquierdo, C
• Del Rio Garma, J
• Guerra Dominguez, L
• Vicuna Andres, I
• Garcia-Arroba Peinado, J
• Zalba Marcos, S
• Vidan Estevez, J M
• Gonzalez Arias, E
• Campuzano Saavedra, V
• Garcia Gala, J M
• Ortega Sanchez, S
• Martinez Nieto, J
• Pardo Gambarte, L
• Sole Rodriguez, M
• Fernandez-Docampo, M
• Avila Idrovo, L F
• Hernandez, L
• Cid, J
• from the Spanish TTP Registry (REPTT, Registro Espanol de la Purpura
• Trombocitopenica Trombotica)

Grupos

• Hematología y Hemoterapia

  

  Investigador Principal

  Javier De La Rubia Comos

Abstract

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 =60years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60years 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 =60years 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 =60years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60years.

© 2023 British Society for Haematology and John Wiley & Sons Ltd.

Keywords

• aged; caplacizumab; rituximab; thrombotic thrombocytopenic purpura