Anemia hemolítica autoinmune: revisión de casos

espanolIntroduccion La anemia hemolitica autoinmune (AHAI) es una enfermedad rara en ninos, generalmente autolimitada. Material y metodos Estudio descriptivo transversal en menores de 18 anos diagnosticados de AHAI desde enero de 1997 a julio de 2019. Se recogieron variables clinicas y se clasificaron segun el test de Coombs directo (TCD) en AHAI por anticuerpos calientes (IgG+/-C3d) y frios (C3d). Se analizo la respuesta al tratamiento y su evolucion. Resultados Se incluyeron 25 pacientes, siendo el 72% varones. La media de edad al diagnostico fue de dos anos (rango 0,4-9). Los sintomas predominantes fueron fiebre (72%), palidez (68%), ictericia (64%), hepatoesplenomegalia y coluria (48%). La mediana de hemoglobina al diagnostico fue 5,4 g/dL. En el 96% el TCD fue positivo, con deteccion de anticuerpos IgG en el 76%. Un solo caso presento TCD negativo. Un 20% asociaron otra citopenia, uno de ellos fue diagnosticado posteriormente de inmunodeficiencia variable comun. En un 32% se sospecho o documento una infeccion viral concomitante. La mayoria de los casos fueron autolimitados y respondieron a tratamiento con corticoides (72%). Aquellos con respuesta parcial (24%), principalmente los que asociaban otras citopenias, precisaron otras lineas de tratamiento (rituximab, micofenolato, inmunoglobulinas). Se detectaron complicaciones (32%) y recaidas (26%) unicamente en AHAI por anticuerpos calientes. Conclusiones Nuestra serie confirma que la AHAI es una enfermedad muy poco frecuente en la infancia. La mayoria de los casos evolucionan favorablemente, aunque hasta una cuarta parte precisan segundas lineas de tratamiento y casos excepcionales tratamientos muy agresivos. Estos ultimos suelen corresponder a pacientes con mas de una citopenia en la evolucion de la enfermedad. EnglishIntroduction Autoimmune hemolytic anemia (AIHA) is a rare and generally self-limiting disease in children. Material and methods A descriptive cross-sectional study was performed in children under 18 years diagnosed with AIHA from January/1997 to July/2019. Clinical variables were collected and AIHA was classified according to the direct antiglobulin test (DAT) in warm AIHA (IgG+/-C3d) and cold AIHA (C3d). Response to treatment and evolution were analyzed. Results 25 patients were included and 72% were males. The median age at diagnosis was 2 years (range 0.4 to 9). Fever (72%), pallor (68%), jaundice (64%), hepatosplenomegaly and coluria (48%) were the most common presenting symptoms. The median hemoglobin at diagnosis was 5.4 g/dl. DAT was positive in 96%, with detection of IgG antibodies in 76%. A single case presented negative DAT. 20% of the patients associated another cytopenia, one of which was subsequently diagnosed with common variable immunodeficiency. Concomitant viral infection was suspected or documented in 32%. Most of the cases were self-limiting and responded to corticosteroid treatment (72%). Those with partial response (24%), mainly those associated with other cytopenias, required other lines of treatment (rituximab, mycophenolate, immunoglobulins). Complications (32%) and relapses (26%) were detected only in warm AIHA. Conclusions Our case series confirms that AIHA is a very rare disease in childhood. Most cases evolve favorably, although up to a quarter of them require second lines of treatment and, in exceptional cases, they need very aggressive treatments. These latter cases generally correspond to patients who present more than one cytopenia in the course of the disease. Previous article in issueNext article in issue