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Histiocytoses of X

Histiocytoses of X — diseases at which in a bone tissue, lymph nodes, marrow, internals proliferata from macrophagic cells with accumulation of lipids in cytoplasm develop.

Epidemiology
Histiocytoses of X meet seldom. The eosinophilic granuloma relating to them is diagnosed mainly for boys aged from 2 — 3 till 12 — 14 and for men at the age of 20 — 30; Hend's illness — Schueller — Krischena is observed at people of all age, is more often at children; Letterer's illness — Siwa comes to light at children aged till 6 months.

Etiology and pathogeny
The etiology of histiocytoses of X is unknown. Opinions on their neoplastic nature or infectious genesis are expressed (medical effect of therapy by antibiotics, the course of a disease after viral infections, detection of a disease at several members of one family, etc. is frequent). At these processes of a proliferata of macrophagic cells infiltrirut many bodies and fabrics. The considerable hyperplasia of macrophages is followed by strengthening of some of their functions — accumulation of lipids, stimulations of lymphocytes to synthesis of the factor promoting development of an eosinophilia.

Classification
The eosinophilic granuloma known as Taratynov's illness, Hend's diseases — Schueller — Krischena and Letterer — Siwa belong to histiocytoses of X. There is an opinion that they represent forms of uniform process with possible transition of one to another.

Approximate formulation of the diagnosis:
1. An eosinophilic granuloma with an insignificant leukocytosis and an eosinophilia in blood, the centers of intra bone destruction of a calvaria, a basin, long tubular bones; defeat of lymph nodes, a liver, lungs, a stomach, skin with a pain syndrome.
2. Letterer's illness — Siwa with gepato-and a splenomegaly, a hyperadenosis, local tumors of bones, anemia, a thrombocytopenic hemorrhagic syndrome.

Clinic
The eosinophilic granuloma in comparison with other forms of a histiocytosis of X differs in rather high-quality current. At damage of several bones or bodies the disease proceeds adversely. Illness duration from several months to several years.

Bones of a skull, basin, edge, long tubular bones are surprised mainly; also extra bone localizations — in lymph nodes, a liver, kidneys, a bladder, a brain, gums, skin, lungs, a stomach meet. Cases of the combined damages of bones and internals are described.
Distinguish multiple, is more often a solitary form of a disease which can be one of process generalization phases. Clinically they are characterized by the constant aching pains in the field of the defeat centers amplifying at the movement. The indisposition, subfebrile condition, a moderate leukocytosis with an eosinophilia, increase of SOE can be observed. There are cases of an asymptomatic current when the centers of defeat are found only at X-ray inspection of bones (defects of homogeneous structure, destruction of an internal cortical plate outside less often, the centers of destruction of 10 in size — 30 mm come to light). In a recovery stage the center of destruction burgeons connecting fabric with the subsequent formation of the new bone having normal structure.

At an eoziiofilny granuloma the increased intra bone proliferation of histiocytes and eosinophilic granulocytes in the form of small groups or extensive fields comes to light. Among them large multinucleate cells meet ugly kernels.

The classical clinical picture of an illness of Hend Schueller — Krischena is provided by an exophthalmos, not sugar mocheiznureniye, damage of bones, a gepatosplenomegaliya (25 — 50%), a limfoadenopatiya. In an acute phase fever, painful infiltrates in various parts of a skeleton, a polydipsia and a polyuria, dermatitis, dedentition are noted. Owing to a specific infiltration of eye-sockets and the Turkish saddle appears pucheglazy, haunch, femoral, humeral bones, edges, a backbone, alveolar departments of a mandible with replacement of teeth from alveoluses can be surprised. There are painful pases pulo-pustular yellowy-brown elements on a face, around eyes, a mouth, on a trunk, in a crotch. In a mouth, under language, in a throat and a throat fibrotichesky defeats are found. The intersticial pulmonary infiltration leads to fibrosis, "with a cellular lung" and to episodes of spontaneous pheumothorax. The pulmonary heart and insufficiency of a right ventricle can develop. Increase in a liver, spleen, lymph nodes develops gradually. Gastrointestinal tract diseases can simulate stomach ulcer or a duodenum, polyps or even a carcinoma. Anemia develops not always, is quite often combined with leucio-and thrombocytopenia. The myelophthisis is sometimes observed. At this form of a histiocytosis of X in macrophages there are a lot of lipids, generally cholesterol that causes gistoplazma vacuolation; at histochemical researches reveal a sudanofiliya and positive reactions to cholesterol. The forecast is less favorable when developing a disease at early age. The death can be result of pulmonary fibrosis, pheumothorax or other reasons. The periods of spontaneous improvement or even remissions are possible.

Letterer's illness Siwa differs in a rapid fatal current. The most part of lethal outcomes comes in the first two years of life. Owing to preferential proliferation of macrophages the clinic of a disease is characterized gepato-and a splenomegaly, a limfoadenopatiya, local tumors of bones, is frequent with the defects defined radiological; anemia, the decrease in number of thrombocytes which is followed by a hemorrhagic syndrome is found; sometimes in a leukocytic formula reveal a monocytosis.

The exophthalmos and not diabetes mellitus meet less than at Hend's illness — Schueller — Krischena. Skin defeats with a tendency to an ulceration of the papules covered with scales and crusts are located mainly on skin a trunk and the heads. The diagnosis is established on the basis of identification of a specific infiltration by histiocytes in punctates and bioptata of bones, lymph nodes and a liver, in the centers of damage of skin.

Verification of the diagnosis
The complex of the provided clinical, cytologic and radiological data allows to diagnose separate forms of a histiocytosis of X. The found skin changes should be differentiated from seborrheal dermatitis, bone — from osteomyelitis or tumoral process, and radiological changes in lungs — from tuberculosis, histoplasmosis or fibrocysts.

Treatment
At local damages of bones of positive takes it is possible to reach use of small doses of radiation therapy. Corticosteroid hormones promote sometimes involution of defects of a skeleton and visceral defeats. At prevalence of process use also polychemotherapy. One of schemes of the choice is use of Prednisolonum in combination with Cyclophosphanum, vinblastine or Vincristinum in the form of 3 — 4 14-day courses with an interval in 2 — 3 weeks. Full or partial remissions are possible. So, for example, at Letterer's illness — Siwa, according to one authors, at 20 — 50% of patients full remissions develop, others report about partial remissions in 50% of cases.

 
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