Histiocytosis  X

[MIM 604 856]

(Langerhans cells granulomatous disease, Langerhans cell histiocytosis, bone eosinophilic granuloma, Letterer-Siwe Disease, Hand-Schuller-Christian disease, Abt-Letterer-Siwe disease, reticulosis X, reticulo-granulomatosis, histiocytic reticulosis, Hashimoto-Pritzker syndrome)

Prevalence: 1/200,000. Most often osseous tumor disease characterized by the abnormal oligoclonal proliferation of histiocytes of the Langerhans cells type (but containing intracytoplasmic Birbeck granules and presenting at their surface antigenic CD1a markers as well as the S100 protein); the  histiocytosis X Langerhans cells are normally localized only at the level of the skin, malpighian mucosae and lung, but they can infiltrate many tissues and organs (spleen, liver, digestive tract, central nervous system, bone) in case of histiocytosis X. 

Clinical onset is more often between the age of 1 and 3 years; polymorphic and highly variable severity events.

In order of decreasing frequency, the usual locations are at the level of bones, skin, lymph nodes, the petrosal bone and mastoid, bone marrow, liver and spleen, the post-hypophysis, and the central nervous system. According to the location, different clinical pictures have been described:

-        Hashimoto-Pritzker auto-involutive cutaneous hHistiocytosis, affecting the newborn, more often with a spontaneously favorable evolution ;

-        Letterer-Siwe Disease,  acute systemic form of the infant, characterized by extensive skin lesions and organ impairment, and of rapidly fatal evolution in the absence of treatment; the treatment is based on the association of corticosteroids and vinblastine

-        Hand-Schuller-Christian disease, involving cranial deficiencies, diabetes insipidus and exophthalmos; it starts between the age of 2 and 5 years and evolves chronically for years, with important sequelae;

-        osseous eosinophilic granuloma,  most often isolated, in older children and the evolution of which is almost always favourable.


Anesthetic implications:

Depending on the clinical presentation, possible pituitary failure.


References:

-        Kallesh A, Kumar VS.
Congenital Langerhans-cell histiocytosis.
NEJM 2021 ; 385 :24, e86


Updated: December 2021