Successful treatment with rituximab in boy with Evans syndrome - case report

Abstract

Autoimmune haemolytic anaemia (AIHA) is caused by production of antibodies against antigens on the surface of the red bloodcells. It can be classifi ed as haemolytic anaemia due to warm and cold reactive antibodies and divided into primary and secondaryAIHA (associated with an underlying disease: systemic lupus erythematodes, immunodefi ciency, lymphoproliferative disorders). Themain feature of this anaemia is positive direct Coombs test which demonstrates antibodies of IgG class and complement (mostlyanti-C3) on the surface of the red blood cells. Evans syndrome is the presence of simultaneous or sequential direct Coombs-positiveAIHA in conjunction with immune thrombocytopenia (ITP). We report on a 2-year-old boy with diagnosed AIHA at age of 10 monthswith a repetitive course. At age of 17 months concomitant ITP occurs and diagnosis of Evans syndrome was made.The patient was treated with glucocorticoids, human immunoglobulins, red blood cells and platelet transfusions many times, withoutthe stabile remission. Finally, treatment with rituximab was followed by clinical improvement and disease remission lasting for months.Key words: autoimmune haemolytic anaemia, immune thrombocytopenia, Evans syndrome, rituximab