Langerhans cell histiocytosis overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Langerhans Cell Histiocytosis (LCH) is a rare disease involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystemic disease.

The disease is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas.

Langerhans Cell Histiocytosis used to be called histiocytosis X, until it was renamed in 1985 by the Histiocyte society[1]

References

  1. "Histiocytosis syndromes in children. Writing Group of the Histiocyte Society". Lancet. 1 (8526): 208–9. 1987. PMID 2880029.


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