HISTIOCYTOSES (histiocytosis, singular; a histiocyte[s] + - osis) — group of the diseases of not clear etiology which are followed by endogenous disturbances of metabolism and accumulation in histiocytes of products of the broken exchange. Treat group G.: the acute system progressing G. (Letterer's disease — Siwa), hron, the system progressing G. (Hend's disease — Schueller — Krischena), carried to systemic limforetikulyarny lesions, and an eosinophilic granuloma. Many researchers consider all three forms G. as the interconnected options (or syndromes) the uniform nosological form designated as proliferative and exchange retikulogistiotsitoz.
The etiology and a pathogeny are not finalized, nosological accessory remains disputable: the polyetiological basis G.
Naiboley a frequent form G. is possible the eosinophilic granuloma is, edges N. I was described. Taratynov in 1913, then it was described by Finzi (O. of Finzi) in 1929. The name of this type of G. was given by L. Likhtenstayn and Jaffa (H. L. Jaffe) only in 1940. In 1944 these authors for the first time applied the term «histiocytosis», combining these on nyatiy three mentioned forms and assuming the inflammatory nature of process. In 1953 L. Likhtenstayn suggested to designate these forms as histiocytoses of X since nosological accessory of process is definitely not established. A part of researchers carries histiocytoses X to reticuloses accumulation or to reactive granulematozny processes, other authors consider all histiocytoses of X as tumors of limforetikulyarny fabric.
The opinion on transformational options of a histiocytosis of X is expressed. So, the eosinophilic granuloma can develop against the background of hron, a system histiocytosis of X, and an acute system histiocytosis of X — to be shown as an aggravation hron, a system histiocytosis of X. It is noted also that at an eosinophilic granuloma perhaps spontaneous increase of fibrosis, transformation of histiocytes (see. Histiocytes ) in lipophages and transformation of an eosinophilic granuloma to lipidic.
Hend's disease — Schueller — Krischena and Letterer's disease — Siwa approach nek-ry endogenous tezaurismozam (see), in particular with sphingolipidoses (see), to the Crimea the disease to Gosha belongs (see. to Gosha disease ) and Nimann's disease — Peak (see. Nimanna — Peak a disease ). Began to rank other similar syndromes as G. — the so-called multitsentrichny retikulogistiotsitoza different from three classical types G. and followed by the peculiar proliferation of histiocytes leading to the accruing fibrosis of bodies.
Eosinophilic granuloma it is observed preferential at the advanced children's and youthful age in the form of local, solitary or multitsentrichny damage of bones, is more rare in the form of combined bone and visceral, is even more rare in the form of the isolated visceral defeats. At visceral forms defeats are frequent went. - kish. a path in the form of the localized knotty or diffusion eosinophilic infiltrates. The system eosinophilic granulomatosis is possible (see. Granulomatoses ). Microscopically it is characterized by focal proliferation of the peculiar fabric consisting of reticular macrophages, leukocytes with eosinophilic cytoplasm, cytologic identical to eosinophilic granulocytes of blood, and multinucleate colossal cells. In late phases of process ksantomny cells appear. The described picture allowed to call this form of a histiocytosis the X retikulogranulemy. In more detail clinic, the forecast and treatment see. Eosinophilic granuloma of bones.
The acute system progressing histiocytosis arises more often on the first year of life. The disease proceeds sharply, is followed by fever, hemorrhagic rash, a cholesterinemia, gepato-and a splenomegaly, increase limf, nodes. At microscopic examination find diffusion infiltration of bodies large atypical one-nuclear reticular macrophages and histiocytes with impurity of multinucleate colossal cells; histiocytes usually do not contain lipids (in this connection this form of a histiocytosis of X is called a nonlipid retikulogistiotsitoz); occasionally they become foamy and contain cholesterol. In more detail clinic, the forecast and treatment see Letterer — Siwa a disease.
The chronic system progressing histiocytosis differs in more high-quality hron, a current. Occurs usually on 4 — the 5th year of life, occasionally at adults, it is characterized by a hypercholesterolemia, gepato-and a splenomegaly, not diabetes mellitus progressing pucheglaziy and focal destruction of flat bones. Disturbance is the cornerstone of process cholesteric exchange (see) that conducts to a visceral xanthomatosis with dystrophy of a bone tissue and internals in connection with adjournment of cholesterol and holesterinester, and also neutral fat and phosphatides in the proliferating histiocytes taking a foamy form and turning in multinuclear huge macrophages. Around the centers xanthomatosis (see) there is a granulematozny reaction and fibrous fabric develops. In an initial stage of process infiltrate consists preferential of eosinophils in this connection some researchers consider an eosinophilic granuloma as a phase of a disease of Hend — Schueller — Krischena. In more detail clinic, the forecast and treatment see. Henda — Schueller — Krischena a disease .
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