Langerhans' cell histiocytosis (histiocytosis X): Immunophenotype and growth fraction

Christine Hage, Cheryl L. Willman, Blaise E. Favara, Peter G. Isaacson

Research output: Contribution to journalArticlepeer-review


The immunophenotype and proliferation fraction have been investigated in 26 cases of Langerhans' cell histiocytosis (LCH). In all cases LCH cells were positive for S-100 protein, CD1a, or both. In most cases LCH cells expressed the macrophage-associated marker CD68 and in two cases they contained lysozyme. Expression of both cytoplasmic CD2 and CD3 was observed in cryostat sections. An unexpected finding was the presence of placental alkaline phosphatase in LCH cells. Langerhans' cells in normal skin were negative for both CD2 and CD3, but a proportion contained placental alkaline phosphatase. In four cases of Rosai-Dorfman disease the histiocytic cells, which share certain immunophenotypic properties with Langerhans' cells, also were positive for placental alkaline phosphatase. A significant proportion of LCH cells stained positively with the antibody to proliferating cell nuclear antigen and also with the proliferation marker Ki-S1. A good correlation between the percentage of Ki-67-positive and proliferating cell nuclear antigen- and Ki-S1-positive cells, respectively, was observed. Thus, in comparison with their putative precursors, LCH cells have an aberrant phenotype and are proliferating locally. This might suggest that LCH is a neoplastic rather than a reactive process.

Original languageEnglish (US)
Pages (from-to)840-845
Number of pages6
JournalHuman Pathology
Issue number8
StatePublished - Aug 1993


  • histiocytosis X
  • immunohistochemistry
  • Langerhans' cell histiocytosis
  • placental-type alkaline phosphatase
  • proliferation fraction
  • Rosai-Dorfman disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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