ACQUIRED AUTOIMMUNE THROMBOCYTOPENIA POST-BONE MARROW TRANSPLANTATIONFOR SEVERE COMBINED IMMUNODEFICIENCY

Children with severe combined immunodeficiency (SCID) have profoundly diminished humoral and cellular immunity resulting in death during inf ancy unless immune reconstitution occurs by bone marrow transplantatio n (BMT). Thrombocytopenia post-bone marrow transplantation can be seen in relation to infection, graft-versus-host disease (GVHD) and rarely , as an autoimmune phenomenon due to immune dysregulation. We report t wo cases of severe AITP following BMT for SCID. Both cases developed l arge intracerebral hemorrhages from which one died. Autoimmune thrombo cytopenia in this setting can be life-threatening and we recommend ear ly and active intervention.