Letterer-Siwe Disease and the Use of Thalidomide for the Treatment

Authors: Colpas PT*, Cintra ML and de Souza EM

Volume 2, Issue 1

Abstract

Langerhans cell histiocytosis is a proliferative, reactive disease, characterized by clonal accumulation of abnormal dendritic cells of bone marrow origin. This work describes a case of a four-month old African-American boy showing clinical, histological, immunohistochemical and electron microscopy evidence for the diagnosis of the Letterer-Siwe disease. He was treated with betamethasone and thalidomide, with relatively good control of the disease but, after the corticoid was withdrawn, clinical symptoms were aggravated, suggesting the patient should be transferred to the local pediatric oncology service, for chemotherapic treatment.

Keywords: Histiocytosis; Langerhans-Cell; Letterer-Siwe Disease; Betamethasone; Thalidomide

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