Purpose: Langerhans cell histiocytosis (LCH) is a disease with a variable clinical manifestation, being localised (SS) or disseminated (MS). The etiology and pathogenesis of LCH is unknown. It is a proliferative disorder of monoclonal origin, but not necessarily neoplastic. In our study we evaluated histopathological parameters and proliferative activity in LCH.
Materials And Methods: Infiltrates from 43 patients with LCH were investigated (nSS=32, nMS=11). We evaluated different histopathological parameters semiquantitatively, demonstrating proliferation using immunohistochemistry for Ki-67.
Results: Overall, the histopathological picture of LCH was heterogeneous. The degree of eosinophilia and presence of necroses was significantly higher in SS-infiltrates compared to MS-infiltrates. Mitotic figures were detected in more than half the infiltrates. The Langerhans origin was verified by CD1a. Ki-67 was highly expressed in all but one infiltrate.
Conclusions: The presence of necrosis and the degree of eosinophilia are related to SS-disease in our study. Ki-67 expression and the presence of mitotic figures indicate that local proliferation contributes to the accumulation of Langerhans cells. Supported by the histopathological appearance of the lesions and a level of Ki-67 expression lower than that of neoplastic tissue, we suggest that LCH is a reactive condition, possibly induced by immunostimulation caused by unknown agents.
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