Histiocytosis X

Histiocytosis X is a group of diseases of unknown etiology with a common pathogenesis, which is based on the reactive proliferation of histiocytes with the accumulation of products of impaired metabolism in them.

Etiology and pathogenesis are unknown. It is believed that the basis is an immunopathological process that promotes focal or disseminated proliferation of histiocytes.

There are three forms of histiocytosis X:

1. Abt-Letterer-Siwe disease is more common in young children and is acute with fever, skin rashes, hepatosplenomegaly, lymphadenopathy, and damage to the lungs and bones.

2. Hand-Schüller-Christian disease is observed at any age and is characterized by defects in the bones of the skull and pelvis, exophthalmos, and diabetes.

3. Taratynov's disease (eosinophilic granuloma) - more often in school-age children, manifests itself with weakness, bone pain, and increased ESR.

The diagnosis is made on the basis of clinical and laboratory data, radiography, and biopsy.

Treatment is carried out with glucocorticoids and cytostatics. The prognosis is better for Hand-Schüller-Christian and Taratynov diseases.

Histiocytosis X (Histiocellular reticulosarcomatosis) is a rare systemic immunoproliferative malignancy that unites a group of oncological diseases with a predominant lesion of bone marrow hematopoiesis, lymphatic system and skin in children