acid-phosphatase and Histiocytosis--Langerhans-Cell

Skin biopsies and a lymphnode of three children with infantile Histiocytosis-X (Letter-Siwe Disease) were studied with enzymehistochemical and sheep-erythrocyte rosetting techniques. The majority of cells making up the infiltrates of skin and lymphnode showed rather weak acid phosphatase and nonspecific esterase activity but considerable leucyl-beta-naphtylamidase activity. Sheep-erythrocyte rosetting techniques performed on frozen sections indicated the presence of receptors for the Fc fragment of IgG, but no receptors for C3 could be demonstrated. Cells with the same enzymehistochemical characteristics could be found in thymus-dependent areas of normal spleen, of normal and reactive lymphnodes and in thymic medulla but not in B-cell areas or thymic cortex. It is suggested that Histiocytosis-X cells belong to the Mononuclear Phagocyte System and that they are related to or identical with cells normally present in the thymus dependent areas of the lymphoid tissue involved with the functioning of cell-mediated immunity.

Topics: Acid Phosphatase; Adenosine Triphosphatases; Complement C3; Erythrocytes; Esterases; Histiocytes; Histiocytosis, Langerhans-Cell; Humans; Immunoglobulin Fc Fragments; Immunoglobulin G; Infant; Langerhans Cells; Leucyl Aminopeptidase; Lymphoid Tissue; Peroxidases; Phagocytes; Rosette Formation; Skin

The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzyme histochemistry for acid phosphatase (ACPase). The major cell type in the lesions was found to express the same Ia+, HTA-1+ phenotype as normal epidermal Langerhans' cells (LC) and was unreactive for ACPase. Additional cell types included Ia-, HTA-1- multinucleate giant cells and residual lymphoid populations. These findings endorse previous concepts that H-X is a proliferation of abnormal LC and emphasise the heterogeneous nature of the cells involved in the disease.

Topics: Acid Phosphatase; Adenosine Triphosphatases; Antibodies, Monoclonal; Antigen-Antibody Complex; Epitopes; Fluorescent Antibody Technique; Histiocytosis, Langerhans-Cell; Histocompatibility Antigens Class II; HLA-D Antigens; Humans; Lymph Nodes; Skin

Topics: Acid Phosphatase; Adolescent; Alkaline Phosphatase; Child; Child, Preschool; Female; Glycogen; Histiocytosis, Langerhans-Cell; Humans; Infant; Leukocyte Count; Male; Monocytes; Naphthol AS D Esterase; Neutrophils; Peroxidases

Characteristic mononuclear cells with distorted nuclei and abundant pale-staining cytoplasms together with osteoclast-like giant cells are the cytologic hallmarks of histiocytosis X. The demonstration of acid phosphatase within giant cells in paraffin-embedded sections is a valuable aid to diagnosis. The histologic differential diagnosis of histiocytosis X includes some allergic granulomas, Hodgkin's disease, myelomonocytic leukemia, mastocytosis, and malignant histiocytosis. Some technical prerequisites for accurate diagnosis of histiocytosis X are considered briefly.

Topics: Acid Phosphatase; Diagnosis, Differential; Histiocytosis, Langerhans-Cell; Histocytochemistry

Topics: Acid Phosphatase; Cell Membrane; Esterases; Histiocytes; Histiocytosis, Langerhans-Cell; Humans; Immunoglobulin G; Phagocytosis

Topics: Acid Phosphatase; Alkaline Phosphatase; Anemia; Bone Marrow Examination; Collagen Diseases; Esterases; Histiocytosis, Langerhans-Cell; Histocytochemistry; Iron; Microscopy, Electron; Mononuclear Phagocyte System