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Expression of Fas ligand in Langerhans' cell histiocytosis: A case report of a boy with multisystem involvement
Author(s) -
Peng ChingTien,
Chow KuanChih,
Chang WuChung,
Tsai ChangHai,
Lin TzeYi,
Lin ShengShing,
Chiu ChanFang
Publication year - 1999
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/(sici)1096-8652(199908)61:4<256::aid-ajh6>3.0.co;2-3
Subject(s) - langerhans cell histiocytosis , pathology , medicine , cd68 , bone marrow , histiocytosis , langerhans cell , peripheral blood mononuclear cell , immunophenotyping , immunohistochemistry , antigen , biology , immunology , biochemistry , in vitro , disease
Previous reports of patients with Langerhans' cell histiocytosis have shown characteristics of osteolytic lesion, visceral involvement and organ dysfunction. We report a 2‐year‐old boy who was diagnosed as Langerhans' cell histiocytosis with a prominent hepatomegaly. X‐Radiogram, computed tomography and magnetic resonance imaging revealed the osteolysis of the right iliac bone, the absence of the left eighth rib as well as the right mandible, and an enhancing mass in the left cerebellum. The data of radiography were highly suggestive of Langerhans' cell lineage. The presence of large CD1a‐positive mononuclear cells associated with inflammatory cells in peripheral blood smear and bone marrow aspirate further confirmed the diagnosis. In addition, expressions of S100, CD25, CD68, CD80, CD86, and Fas ligand were identified on these cells by immunocytochemical staining. The results indicate that although these cells are activated Langerhans' cells, progression of the disease and the bone destruction could be mediated by the overt FasL expression of the cells. Am. J. Hematol. 61:256–261, 1999. © 1999 Wiley‐Liss, Inc.