alpha-chymotrypsin and Histiocytosis--Langerhans-Cell

Recent evidence suggests that the proliferative cells of idiopathic histiocytosis may be derived from Langerhans cells. In this study, antisera to S-100 protein, HLA-DR (la-like) antigen, muramidase, and alpha 1-antichymotrypsin were tested on formalin-fixed, paraffin-embedded tissue from nine cases of idiopathic histiocytosis using an immunoperoxidase technique. Tumor cells were positive for S-100 protein and HLA-DR antigen but negative for muramidase and alpha 1-antichymotrypsin. Mononuclear phagocytes were positive for HLA-DR antigen, muramidase, and alpha 1-antichymotrypsin but negative for S-100 protein. The immunohistochemical staining pattern of the tumor cells in these cases of idiopathic histiocytosis is similar to that seen for normal Langerhans cells. When these results are coupled with electron microscopic and histochemical data, it would appear that the origin of cells in idiopathic histiocytosis is from the Langerhans cell or its precursor. Thus, this condition might be better designated "Langerhans cell disease."

Topics: alpha 1-Antichymotrypsin; Chymotrypsin; Histiocytosis, Langerhans-Cell; Histocompatibility Antigens Class II; HLA-DR Antigens; Humans; Immunoenzyme Techniques; Langerhans Cells; Mandibular Diseases; Maxillary Diseases; Muramidase; Phagocytes; S100 Proteins; Staining and Labeling

Topics: alpha 1-Antichymotrypsin; Biopsy; Chymotrypsin; Diagnosis, Differential; Histiocytes; Histiocytosis, Langerhans-Cell; Histocytochemistry; Humans; Immunochemistry; Immunoglobulins; Lymph Nodes; Muramidase; Phagocytosis

The cellular nature of the proliferating histiocytes in 6 cases of histiocytosis X was studied immunohistochemically and ultrastructurally. Immunohistochemically, S-100 protein was detected both in the cytoplasm and the nuclei of histiocytosis X cells as well as Langerhans cells in normal oral epithelium. These cells were always negative for lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and immunoglobulins. S-100 protein was not detected in lysozyme-positive histiocytes and multinucleated giant cells often showed the signs of phagocytosis. Thus, S-100 protein appears to be a useful immunohistochemical marker for histiocytosis X cells. Ultrastructurally, Birbeck granules noticed in histiocytosis X cells were never seen in the phagocytic histiocytes with numerous lysosomes and phagosomes. These results emphasized the heterogeneous nature of the proliferating histiocytes involved in the lesions. Since histiocytosis X cells share characteristics, not only ultrastructurally but also immunohistochemically, with Langerhans cells, the hypothesis that histiocytosis X may be fundamentally an abnormal proliferation of Langerhans cells has been further supported.

Topics: alpha 1-Antichymotrypsin; alpha 1-Antitrypsin; Cell Division; Chymotrypsin; Histiocytes; Histiocytosis, Langerhans-Cell; Histocytochemistry; Humans; Immunoenzyme Techniques; Immunoglobulins; Muramidase; S100 Proteins

Topics: alpha 1-Antichymotrypsin; Chymotrypsin; Diagnosis, Differential; Histiocytes; Histiocytoma, Benign Fibrous; Histiocytosis, Langerhans-Cell; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase