Unusual immunophenotype displayed by histiocytes in haemophagocytic lymphohistiocystosis.
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.
Herlin
T
T
Department of Pediatrics, University Institute of Pathology, Aarhus, Denmark.
Pallesen
G
G
Kristensen
T
T
Clausen
N
N
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
D000911:Antibodies, Monoclonal; D000954:Antigens, Surface