Histiocytosis X

Histiocytosis X is a group of diseases of unclear etiology with a common pathogenesis, which is based on the reactive proliferation of gamciocytes with the accumulation of metabolic products in them.

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

Clinical picture. There are three forms of histiocytosis X: Abta-Letterer-Sieve disease, Hend-Schüller-Krissen's disease (xanthomatosis), Taratinov's disease (eosinophilic granuloma), differing in clinical picture and prognosis. Perhaps all three forms are variants of one disease; Their mutual transitions can be observed.

Abt-Letterter-Sieve disease is more common in young children. It develops sharply (rarely gradually) with high fever, skin rashes (papules in the sternum, spine with yellowish crusts, hemorrhages, mocclusion, seborrhea), hepatosplenomegaly, generalized enlargement of the lymph nodes, otitis and / or mastoiditis, lung damage (interstitial pneumonia), Defeat of flat bones, diabetes insipidus, exophthalmos, elevated ESR, neutrophilic leukocytosis. On the roentgenograms, destructive changes in the bones.

Hend-Schüller-Crischen's disease affects children of any age. Typical defects of the bones of the skull or / and pelvis, exophthalmus, diabetes insipidus. There may be a combination of other signs, which is associated with the primary lesion of these or other organs: obesity, lag in physical development, hepatomegaly, lymphadenopathy, petechial rash, seborrhea, changes in the lungs, stomatitis. The blood determines leukocytosis, eosinophilia, an increase in the number of reticular and plasma cells, increased ESR, hypercholesterolemia, hypoalbuminemia, hyperglobulinemia, hyperbetalipoproteinemia. Often joins a secondary infection.

The disease of Taratynov (eosinophilic granuloma) is observed mainly in children of school age. Typical: general weakness, increased fatigue, decreased appetite, bone pain (both flat and tubular bones are affected), increased ESR, and sometimes eosinophilia. In some cases, the disease is asymptomatic and ends with spontaneous cure. Radiograms of bones reveal foci of destruction, more often of round or oval form without sclerosis zones. In a small number of cases, the clinical picture of the disease is brighter: diabetes insipidus, exophthalmos, hepato- or hepatosplenomegaly, anemia, various skin changes, etc.

The diagnosis is based on clinical and laboratory data, myelograms, skin biopsies and lymph nodes, in which hyperplasia of the reticular tissue, eosinophils, xanthoma cells is revealed. Differential diagnosis should be carried out with osteomyelitis, bone form of tuberculosis, osteosarcoma, neuroblastoma, fibrotic osteodystrophy, lymphogranulomatosis, leukemia, glycolipidosis (Gaucher's disease and Niman-Pick disease), portal hypertension.

Treatment, as a rule, is spent in a hospital. In acute manifestations, glucocorticoids are administered in combination with cytostatics (vincristine, leukeran, chlorobutin, etc.). Prednisolone in a dose of 40 mg per 1 square. M of the child's body surface is given daily for 2 weeks. Vincristine -1.5 mg / square. M / in once a week or leukeran - 0.1 mg / kg daily for 2 weeks, then a break 2 weeks. Such cycles of treatment are carried out to 10. With Hend-Schuller-Krischen and Taratynov diseases, if there is no generalized lesion of internal organs, less massive therapy is performed. In all cases, the main therapy is combined with symptomatic (drugs of the posterior lobe of the pituitary gland, hypothiazide, vitamins). There are indications of the efficacy of decaris (levamisole) and thymosin (timapine).

The prognosis for Hend-Schüller-Krischen and Taratynov diseases is more favorable than in the case of Abta-Letterter-Siwa disease and is determined by the timely treatment started.