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Poster Networking

Acquired and Critical Care Bleeding

PB1020 - Acquired Haemophilia Spanish registry, characteristics of infectious complications after 10 years activity

Tuesday, June 25, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall

    Presenting Author(s)

  • Maria-Eva Mingot-Castellano, MD, PhD

    Hematologist
    Hospital Universitario Virgen del Rocio, Instituto de Biomedicina de Sevilla, Spain
    Sevilla, Andalucia, Spain

    • Co-Author(s)

  • SH

    Saturnino Haya

    Hematologist
    Hospital Universitario La Fe, Valencia
    Valencia, Comunidad Valenciana, Spain

  • JP

    Jose Pardos-Gea

    Internal Medicine
    Hospital Universitario Vall d´Hebrón
    Barcelona, Catalonia, Spain

  • AM

    Ana Marco-Rico

    Hematologist
    Departamento de Hemostasia y Trombosis, Hospital General Universitario Dr. Balmis, Alicante, Spain
    Alicante, Comunidad Valenciana, Spain

  • José M Bastida

    Hematologist
    Departamento de Hematología, Complejo Asistencial Universitario de Salamanca (CAUSA), Instituto de Investigación Biomédica de Salamanca (IBSAL), Centro de Investigación del Cáncer, IBMCC-CSIC, Universidad de Salamanca (USAL), Salamanca, Spain. #Grupo Español de Alteraciones Plaquetarias Congénitas (GEAPC).
    Salamanca, Castilla y Leon, Spain

  • MC

    Mariana Canaro

    Hematologist
    Hospital de Son Espases
    Palma de Mallorca, Islas Baleares, Spain

  • AB

    Angel Bernardo Gutierrez

    Hematologist
    Hospital Universitario Central de Asturias
    Oviedo, Asturias, Spain

  • AR

    Agustín Rodríguez-Alen

    Hematologist
    Servicio de Hematología, Hospital Universitario de Toledo, Toledo, Spain
    Toledo, Castilla-La Mancha, Spain

  • BG

    Belén González Mesones

    Hematologist
    Hospital Universitario Marqués de Valdecilla
    Santander, Cantabria, Spain

  • FG

    Faustino Garcia Candel

    Hematologist
    Hospital Clínico Universitario Virgen de la Arrixaca
    Murcia, Murcia, Spain

  • DT

    Dolors Tassies Penella

    Hematologist
    Hospital Clinic
    Barcelona, Catalonia, Spain

  • ER

    Elena Rosello Palmer

    Hematologist
    Hospital Universitari de Belvitge
    Barcelona, Catalonia, Spain

  • Cristina Sierra Aisa, MD

    Hematologist
    Servicio de Hematología, Hospital Universitario de Cruces, Baracaldo, Spain
    Barakaldo, Pais Vasco, Spain

  • MC

    Marina Carrasco Expósito

    Hematologist
    Sant Pau Campus Salut Barcelona
    Barcelona, Catalonia, Spain

  • SA

    Susana Asenjo

    PhD MD
    Servicio de Hematología, Hospital Clínico San Carlos,
    Madrid, Madrid, Spain

  • MF

    Maria Carmen Fernandez Sanchez de Mora

    Hematologist
    Hospital Universitario Reina Sofia
    Córdoba, Andalucia, Spain

  • MA

    MARIA NIEVES ALONSO ESCOBAR

    Hematologist
    Complejo Hospitalario Universitario, Badajoz
    Badajoz, Extremadura, Spain

  • María Teresa Alvarez-Román, MD, PhD

    Head of the Haemostasis Unit
    Department of Hematology, University Hospital La Paz, Autonoma University of Madrid, Madrid, Spain
    Madrid, Madrid, Spain

  • RL

    Rafael Lluch Garcia

    Hematologist
    Hospital Universitario de la Ribera
    Valencia, Comunidad Valenciana, Spain

  • PM

    Pascual Marco Vera

    Hematologist
    Hospital General Universitario de Alicante
    Alicante, Comunidad Valenciana, Spain

Background: Acquired hemophilia (AHA) is a rare bleeding disorder caused by autoantibodies against coagulation factors. It is necessary to establish hemostatic treatment and to eliminate the inhibitor using immunosuppressant drugs. Infections are frequent iatrogenic adverse events in these patients.

Aims: To describe the clinical characteristics and response to treatment of AHA according to Spanish registry and to define outcomes and infectious complications.

Methods: In 2014 the Spanish registry of acquired hemophilia was activated. It is located on the SETH (Spanish Society of Thrombosis and Haemostasis) website. This registry has been implemented according to all national requirements and the Helsinki Declaration.

Results: 233 patients are registered from 2014 to 2023 (0.55 patients/million habitants/year), 57.5% of them are male. Median age was 73.5 years old (IQR, 63.3-82 years). Mean inhibitor titerwas 24.5BU (IQR, 11.3-63.5BU). 54% of patients had an underliying disease (31.5% autoimmune, 15% neoplasms, 6% postpartum) and 37.6% were under anticoagulant or antiplatelet treatment one month before diagnosis. 89% of patients presented clinically relevant bleeds and 69% of patients needed hemostatic treatment during AHA (35% rFVIIa, 14.2% aCCP, 20% both). Emicizumab was used in 3 patients under different patterns. Regarding to inhibitor eradication, 96.4% patients received immunosuppressive treatment (44% steroids plus cyclophosphamide, 13.3% rituximab plus cyclophosphamide, 14%, steroids), median time to achieve complete response was 6 weeks (IQR, 3-12 weeks). With a median follow-up of 13 months (IQR, 2 – 41.5 months), mortality rate was 26.6%, due to infections (46.8%), bleeding (11.3%) or underlaying condition (21%). Related to infections (Table 1, 15.5% of patients needed treatment and/or hospitalization, and 62% were mortal.

Conclusion(s): There were not differences in response rates related to eradication schemes. The first cause of death was infectious diseases, so initiatives to optimize immunosuppressive treatment or infectious prophylaxis are needed.