Autoimmune pancytopenia of childhood associated with multisystem disease manifestations

Abstract

Five patients with immune thrombocytopenia and neutropenia, including three with autoimmune hemolytic anemia, are described; none had identifiable underlying disease. All patients had demonstrable autoantibodies against multiple hematopoietic cell types, and in two patients, the antiplatelet and antierythrocyte antibodies were shown to be distinct. All patients had continuing disease, and with follow-up of 2 to 6 1/2 years, all developed nonhematologic disease manifestations. These patients demonstrate that even in the absence of identifiable underlying disease, autoimmune hematologic disease resulting from production of antibodies against red cells, white cells, and platelets often involves a generalized disorder of immune regulation, with multisystem disease manifestations and a prolonged course.