PERIOSTEAL GAUCHER-LIKE CELLS IN BETA-THALASSEMIA MAJOR

A 24-year-old Chinese woman who has undergone staged surgery for crani ofacial deformity secondary to beta-thalassemia major is presented. Lo cal clusters of Gaucher-like cells were found in the periosteum of the mandible. The histologic and ultrastructural features of these cells are described and the pathogenesis and differential diagnoses discusse d. To the best of our knowledge, this is the first reported case of Ga ucher-like cells occurring outside the lymphohematopoietic system in t halassemic patients.