Autoimmune Hemolytic Anemia in Children, 20 Years Experience of Single Center

Abstract

Aim: Autoimmune hemolytic anemia (AIHA) is a rare disease in pediatrics, whose mortality rate was reported to be as high as 10%.AIHA can be primary or secondary to other diseases, Availability of new immunsupressive drugs like mycofenolate mofetil (MMF),has provided the opportunity to reduce long term steroid administration and mortality. In this study we aimed to represent AIHApatients of 20 years, from single centre and focus on the causes, treatment and outcomes. The secondary object was to representoutcomes of patients who received MMF.Material and Methods: This study was designed as a retrospective study. Patients aged three months to 18 years old with hemoglobinlevel less than 10 g/dl and positive DAT with signs of hemolysis were included in the study.Results: Twenty five AIHA patients (F/ M: 14/ 11) aged 6.2± 4.6 years old were followed- up for a mean period of 5.3± 4.8 years.Primary AIHA was detected in 12 (48%) patients. Immune deficiency/ autoimmune lymphoproliferative syndrome was the prominentetiological factor in secondary AIHA. The other underlying diseases were systemic lupus erythematosus, malignancy, autoimmunehepatitis and infection.Eleven patients received MMF with a mean duration of 2.6± 1.6 years. Two of them had primary AIHA, the others had secondarydisease. During the follow- up time, eight patients (75%) achieved remission with MMF. None of MMF users developed side effect.One but all patients with AIHA achieved remission. No death related to AIHA was recorde': recordedConclusion: Understanding the biology of the disease and making accurate diagnosis is important to avoid harmful treatment andto consider targeted therapy. After the failure of first line therapy with steroids or as a steroid- sparing agent, MMF seems to be aneffective second-line maintanance immunosuppressive drug without significant side effects.