A case of fatal thrombotic thrombocytopenic purpura (TTP) in a pediatr
ic patient with HIV infection is reported. The diagnosis was made at a
utopsy based on the presence of platelet microthrombi in multiple orga
ns, including the cardiac conduction system. The disease presented aty
pically with hemorrhagic manifestations, thrombocytopenia, and hemolyt
ic anemia, without significant accompanying neurologic and renal abnor
malities. Autoimmune-based thrombocytopenia has been well documented i
n the presence of HIV infection in both the adult and the pediatric po
pulation. We believe this to be the first reported case of TTP in an H
IV-positive child.