Abstract
The patient was a 36 year-old male. Hepatosplenomegaly appeared at 6 months and systemic lymph node swelling, anemia, leukopenia and thrombocytopenia appeared at 18 months. At 3 years, hemorrhagic spots appeared. The Letterer-Siwe disease was diagnosed from a lymph node biopsy. Remission was achieved by administration of corticosteroids and blood transfusion, and the clinical course of the patient was observed for 33 years until he died of liver cirrhosis caused by post-transfusion hepatitis. The marked proliferation of atypical histocytes in the lymph nodes, seen histologically at 3 years, was alleviated at 16 years, and disappeared after 29 years of age. Letterer-Siwe disease is usually progressive and has a poor prognosis, but in this case, the disease gradually remitted as the patient grew older. Considering the clinical course of this patient, it appears possible that Letterer-Siwe disease may heal and show a good prognosis once it has gone into remission.
