A 19-year-old woman presented with clinical manifestations of sudden, fulmi
nant thrombotic thrombocytopenic purpura associated with autoimmune hepatit
is and autoimmune thrombocytopenic purpura. Although thrombotic thrombocyto
penic purpura may, rarely, be associated with systemic lupus erythematosus
and other autoimmune diseases, to our knowledge, the syndrome has never bee
n described in association with autoimmune hepatitis. In this patient, too,
the etiology of thrombotic thrombocytopenic purpura associated with autoim
mune disease remains elusive. The patient was treated with corticosteroid,
which brought about no improvement in her condition, and she died of multio
rgan failure. Diagnosis is challenging, but prompt diagnosis is necessary b
ecause thrombotic thrombocytopenic purpura is a life-threatening syndrome w
hose prognosis has been improved significantly by early plasmapheresis trea
tment.