[Evans syndrome in a child]

Area of Science:

• Pediatrics
• Immunology
• Hematology

Background:

• Evans' syndrome is a rare autoimmune disorder characterized by concurrent hemolytic anemia and immune thrombocytopenia.
• Childhood-onset Evans' syndrome presents diagnostic and therapeutic challenges, particularly in cases refractory to standard treatments.

Observation:

• A 9-month-old Bedouin girl presented with sequential Coombs-positive hemolytic anemia and immune thrombocytopenia.
• Initial laboratory workup excluded other underlying diseases, leading to a diagnosis of Evans' syndrome.

Findings:

• Corticosteroid therapy initially achieved normal hemoglobin and thrombocyte counts within one month.
• Relapses occurred upon corticosteroid dose tapering, indicating steroid dependency.

Implications:

• High-dose intravenous gamma-globulin was effective in managing relapses, suggesting its utility in refractory childhood Evans' syndrome.
• This case highlights the complex management of pediatric Evans' syndrome and the potential role of alternative therapies.