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Published on: March 26, 2018
Langerhans' cell histiocytosis (histiocytosis X): immunophenotype and growth fraction
C Hage1, C L Willman, B E Favara
1Department of Histopathology, University College London Medical School, UK.
Insights
Langerhans' cell histiocytosis (LCH) cells exhibit an aberrant immunophenotype and local proliferation, differing from their precursors. This suggests LCH may be a neoplastic process rather than reactive.
Area of Science:
- Immunology
- Oncology
- Cell Biology
Background:
- Langerhans' cell histiocytosis (LCH) is a rare disorder.
- The cellular origin and behavior of LCH cells are not fully understood.
- Distinguishing between reactive and neoplastic processes in LCH is crucial.
Purpose of the Study:
- To investigate the immunophenotype and proliferation fraction of LCH cells.
- To compare LCH cells with normal Langerhans' cells and Rosai-Dorfman disease cells.
- To determine if LCH represents a neoplastic or reactive condition.
Main Methods:
- Immunohistochemical analysis of 26 LCH cases.
- Assessment of S-100 protein, CD1a, CD68, lysozyme, CD2, CD3, and placental alkaline phosphatase expression.
- Evaluation of proliferation markers: proliferating cell nuclear antigen (PCNA) and Ki-S1 (Ki-67).
Main Results:
- LCH cells consistently expressed S-100 protein and/or CD1a.
- Aberrant expression of cytoplasmic CD2 and CD3 was observed in LCH cells.
- A significant proportion of LCH cells showed positive staining for proliferation markers (PCNA, Ki-S1, Ki-67).
- Placental alkaline phosphatase was present in LCH cells, unlike normal Langerhans' cells.
Conclusions:
- LCH cells display an aberrant immunophenotype and proliferate locally.
- The findings suggest a neoplastic origin for LCH.
- Further research is warranted to elucidate the precise nature of LCH.
Abstract:
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.
