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Langerhan's Cell Histiocytosis (Histiocytosis X)

Langerhan's cell histiocytosis (LCH) comprises a class of rare childhood syndromes believed to affect the immune system and its regulation. The clinical entities include eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease.

As the class I histiocytoses, LCH is marked by an accumulation or proliferation of Langerhan's cell, an antigen-processing monocytic (white) cell of the skin. The accumulation leads to cellular lesions of bone, skin and other body organs.

Bone is affected in 80 percent of patients, resulting in single or multiple lesions that appear most often in the skull and weight-bearing bones, such as the spine. Fifty percent of patients have skin involvement, generally dermatitis, or lesions on the feet, hands and back.