All issues > Volume 28(8); 1985

Case Report

J Korean Pediatr Soc. 1985;28(8):817-822. Published online August 31, 1985.

Three Cases of the Letterer-Siwe Disease.

M H Kim, E K Choi, M Y Chung, D H Keum

¹Dept, of Pediatrics, Korea General Hospital

Abstract

Letterer-Siwe disease was an established entity with the following fature: occurrence in infancy, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, tumors of bone, generalized hyperplasia of nonlipid storing macrophages in many organs but prominently in skin, and rapidly fatal downhill course. The diognosis was confirmed by clinical symptoms, laboratory data, histopathologic and radiologic findings. During past 6 years, we experienced 3 cases of the disease and 2 of them expired 4 months after the diagnosis established, but the another 1 case was not fully followed-up. A brief review of literature was made.

Keywords :Letterer-Siwe Disease