title: Unusual immunophenotype displayed by histiocytes in haemophagocytic lymphohistiocystosis.
abstract: Extensive immunophenotypic studies in a 2 1/2 month old girl with haemophagocytic lymphohistiocytosis were performed to characterise the proliferating histiocytes of the disease. The cells strongly expressed conventional macrophage antigens, but unexpectedly, there was a dissociated expression of the CD1a antigen (reacting with the monoclonal antibody NA1/34 but not with OKT6) and intracellular S-100 protein by the haemophagocytic lymphohistiocytosis histiocytes. These findings indicate that there is a "hybrid" phenotype between the two main arms of the mononuclear phagocyte system--namely, Langerhans' cells and phagocytic macrophages.
authors.affiliation: Department of Pediatrics, University Institute of Pathology, Aarhus, Denmark.
authors.forename: T
authors.initials: T
authors.lastname: Herlin
authors.forename: G
authors.initials: G
authors.lastname: Pallesen
authors.forename: T
authors.initials: T
authors.lastname: Kristensen
authors.forename: N
authors.initials: N
authors.lastname: Clausen
mesh_terms: D000911:Antibodies, Monoclonal; D000954:Antigens, Surface / Q000032:analysis; D005260:Female; D006644:Histiocytes / Q000276:immunology*; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D008206:Lymphatic Diseases / Q000276:immunology*; D008218:Lymphocytosis / Q000276:immunology*; D010587:Phagocytosis; D010641:Phenotype
chemical_list: D000911:Antibodies, Monoclonal; D000954:Antigens, Surface