Gistiocitozы

For this group of diseases characterized by the presence in the tissues of proliferative histiocytes.

Disease-Henda Shyullera-Christian

Disease-Henda Shyullera-Christian differs from other histiocytosis mainly chemical the composition of deposits in the cells of the mononuclear phagocyte system. As a result of violation of lipid metabolism in blood circulating cholesterol, cholesterol esters, neutral fats, phosphatides and. They are deposited in the cells of the mononuclear phagocyte system, which in this disease dramatically hyperplastic.

With widespread retikuloksantomatoze appear ksantomatoznye cells, identical cells Niemann-Pick. Some of them are huge and multicore. Around the cluster of cells develops ksantomatoznyh granulomatous tissue with foci of fibrosis. So the specific form of this disease granulomas, which can be completely absorbed, and then restores the structure of tissues.

The disease is very rare. Its form is characterized by bone marrow hepatosplenomegaly, diabetes insipidus, progressive exophthalmos and focal destruction of the flat bones. There are other clinical and morphological variants of the disease-Henda Shyullera-Christian.

Nervous Letterer-Gray

The mechanism of the disease are cell proliferation of mononuclear phagocytes in the skin, liver, spleen, lymph nodes, bone marrow, light, bones and other organs. This atypical histiocytes with a medium-sized round or oval nucleus, often contains basophilic nucleolus.

The cytoplasm of the cell is painted in bright colors, bazofnlnaya, Homogeneous. There are also many nuclear histiocytes, as well as clusters of cells ksantomnyh, containing cytoplasmic deposits of cholesterol and neutral fats, plasma cells and eosinophils. The disease begins with acute skin lesions, onset of fever. Then increased spleen, liver and lymph nodes, which often form conglomerates. There are inflammation of the mucous membranes (stomatitis, vulvovaginitis et al.), do not respond to antibiotic therapy. Often there is a loss of flat bones (Skull, and other blades.).

Special specific changes in the blood is not observed. May include the elevated erythrocyte sedimentation rate, neutrocytosis, eozinofilija, monocytic, platelets in severe cases may develop pancytopenia.

The diagnosis of the disease It is placed on the study of puncture and biopsy of the sites above cell proliferation.

Eosinophilic granuloma

Eosinophilic granuloma characterized intraosseous proliferation of histiocytes and eosinophilic granulocytes.