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Unusual immunophenotype displayed by histiocytes in haemophagocytic lymphohistiocystosis.
  1. T Herlin,
  2. G Pallesen,
  3. T Kristensen,
  4. N Clausen
  1. Department of Pediatrics, University Institute of Pathology, Aarhus, Denmark.

    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.

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