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Vol. 160. Issue 1.
Pages 30-38 (January 2023)
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Vol. 160. Issue 1.
Pages 30-38 (January 2023)
Review
Autoimmune haemolytic anaemia
Anemia hemolítica autoinmune
Visits
9
María Argüello Marina
Corresponding author
marargumar@gmail.com

Corresponding author.
, Montserrat López Rubio, Lucía Castilla García
Servicio de Hematología y Hemoterapia, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain
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Abstract

Autoimmune haemolytic anaemias (AIHA) are acquired haematological disorders caused by increased peripheral erythrocyte destruction mediated by autoantibodies against erythrocyte antigens. They classifie according to aetiology into primary and secondary, and according to the type of antibody and reaction temperature into AIHA due to warm antibodies (w-AIHA) and AIHA due to cold antibodies (c-AIHA).

The mainstay of management in w-AIHA remains glucocorticoid therapy, and the early addition of rituximab has shown good results in recent studies. Primary c-AIHA is mainly treated with rituximab, alone or in combination with chemotherapy.

New drugs such as Syk inhibitors, anti-FcRn Ig and complement inhibitors are in advanced development and will expand the therapeutic arsenal, especially in refractory or relapsed cases.

Keywords:
Autoimmune haemolytic anaemia
Autoantibodies
Review
Update
Haemolysis
Anaemia
Erythropathology
Acquired haemolytic anaemias
Direct Coombs
Rituximab
Complement
Resumen

Las anemias hemolíticas autoinmunes (AHAI) son trastornos hematológicos adquiridos ocasionados por una destrucción periférica de eritrocitos incrementada, mediada por autoanticuerpos dirigidos frente a antígenos eritrocitarios. Se clasifican según etiología en primarias y secundarias, y según el tipo de anticuerpo detectado y temperatura de reacción en AHAI por anticuerpos calientes (AHAI-C) y AHAI por anticuerpos fríos (AHAI-F).

El pilar del manejo en AHAI-C continúa siendo el tratamiento con glucocorticoides, y la adición precoz de rituximab ha demostrado buenos resultados en los últimos estudios. Las AHAI-F primarias se tratan principalmente con rituximab, sólo o combinado con quimioterapia.

En fase de desarrollo avanzado encontramos nuevos fármacos como los inhibidores de Syk, Ig anti FcRn e inhibidores del complemento, que permitirán ampliar el arsenal terapéutico, especialmente en casos refractarios o recidivantes.

Palabras clave:
Anemia hemolítica autoinmune
Autoanticuerpos
Revisión
Actualización
Hemólisis
Anemia
Eritropatología
Anemias hemolíticas adquiridas
Coombs directo
Rituximab
Complemento

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