[Autoimmune hemolytic anemia in children. Apropos of 14 cases].
Autoimmune hemolytic anemia is a rare disorder in childhood. The therapeutical difficulties encountered are described in a series of 14 patients aged 6 weeks to 10 years, 8 of them being under the age of 1 year at time of diagnosis. A remission was observed spontaneously in 1 patient and was obtained with steroids in 8 others. One patient died from acute irreversible hemolysis. Four patients were splenectomized because of an immediate or delayed resistance to steroids. After splenectomy, two patients died from infection, one was cured and the last patient is still in remission after one year. Steroid therapy is the primary treatment of autoimmune hemolytic anemia. The initial dose of 2 mg/kg/day has to be maintained until the remission (normal hemoglobin level and reticulocyte count) is achieved, then progressively decreased leading to alternate day therapy for several months. Resistance to steroid requires splenectomy, although it is an hazardous treatment in young children. The indication for immunosuppressive agents in childhood is difficult to define. It can be proposed in patients with steroid dependency in order to reduce the dose of steroid and/or to avoid long term steroid therapy.
D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy*; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012075:Remission, Spontaneous; D013156:Splenectomy; D013997:Time Factors
D000305:Adrenal Cortex Hormones
Anémies hémolytiques auto-immunes de l'enfant. A propos de 14 observations.