Refractory thrombocytopenia as an initial presentation of childhood systemic lupus erythematosus and its response to rituximab.

A previously healthy adolescent girl presented with oral ulcer, skin and mucosal bleeding for 20 days. On evaluation, she was found to have autoimmune haemolytic anaemia, autoimmune thrombocytopenia, low complement levels and positive antinuclear antibodies with very high titres (1:3200). The anaemia responded to intravenous immunoglobulin and intravenous methylprednisolone, but thrombocytopenia was refractory. She received two doses of rituximab, 2 weeks apart, after which the bleeding manifestations subsided and platelet counts normalised. Here, we present a case of childhood systemic lupus erythematosus with autoimmune haemolytic anaemia and refractory thrombocytopenia at the disease onset and its response to rituximab.