Langerhans cell histiocytosis (LCH) is a hemopoietic disorder that can affect single or multiple sites simultaneously. It is a disorder that affects mostly children. The clinical course is variable and can range from isolated disease that resolves spontaneously to systemic disease that is rapidly progressive and lethal.
LCH was originally known as histiocytosis X. There were traditionally three categories: Letterer-Siwe syndrome, Hand-Schuller-Christian disease, and eosinophilic granuloma. Letterer-Siwe syndrome refers to a constellation of symptoms characterized by splenomegaly, hepatomegaly, lymphadenopathy, and bone lesions. Hand-Schuller-Christian disease included diabetes insipidus and exophthalmos in addition to the bone lesions. Eosinophilic granuloma typically involves a single bony lesion. Current terminology of LCH includes chronic unifocal LCH, multifocal LCH, and acute disseminated LCH. It is now known that all three disorders are part of a clinical spectrum of the same underlying disease (1).
Was this article helpful?