HYoNDA — SCHUELLER — KRYSChE-NA the DISEASE (A. Hand, an amer. pediatrician, 1868 — 1949; A. Schuller of t Austrian neuropathologist and psychiatrist, 1874 — 1958; N of A. Christian * amer. doctor, 1876 — 1951; synonym: a lipoid granulomatosis, Krischen's disease — Schueller, a lipogranulomatosis, the chronic system progressing histiocytosis) — the disease from group of histiocytoses of X which is characterized by infiltration of various bodies and fabrics atypical histiocytic elements.
The disease was described for the first time by Hend in 1893; he observed at the three-year-old child of damage of skin, bones of a skull, increase in a liver and spleen. Schueller (1916) and Krischen (1919) allocated a triad of symptoms at this disease: defects of flat bones, an exophthalmos (see) and not diabetes mellitus (see Diabetes not sugar).
In 1953 L. Likhtenstayn offered the term «histiocytoses of X» combining Hend's disease — Schueller — Krischena, a disease of Let-terera — Siwa (see Letterer — With a willow a disease) and an eosinophilic granuloma (see). According to the WHO classification (1976), these diseases belong to the tumoral diseases of the hemopoietic and adenoid tissue entering into subgroup «chronic mo-notsitoidny leukemia and general histiocytic diseases» (see Histiocytoses).
The disease most often occurs at children at the age of 4 — 5 years, is much more rare at adults.
The etiology and a pathogeny are up to the end not studied. A number of researchers refer changes at Hend's disease — Schueller — Krischena to reactive, and a disease — to inf. to diseases, perhaps, virus etiology. Pao (Rao) and sotr. (1970) consider that developing of a disease is promoted by the inadequate answer of an organism to any infection; Nesbit and Bends (M. Nesbit, W. Krivit, 1975) connect a course of a disease with immunol. insufficiency. Other researchers believe that disorder of function of system of mononuclear phagocytes and disturbance of a lipometabolism is the cornerstone of a disease; they carry it to a xanthomatosis (see).
Pathological anatomy. At a macroscopic research in bones, especially in bones of a skull, the multiple defects of various size filled with the soft, sometimes crumbling tumorous granulematozny раз-# rastaniye of yellow-gray color come to light. At' damage of an eye-socket there is an exophthalmos, at damage of bones of a base of skull destruction of a temporal bone, and in some cases the Turkish saddle, a prelum of a hypophysis or distribution to it and hypothalamic area of granulematozny growths is possible. During the involvement in patol. process of c. the N of page is observed a thickening of a meninx and infiltration of substance of a brain. In lungs small knots and tyazh of granulematozny growths are found, to-rye are located subplevralno, around vessels and bronchial tubes, on the course of interalveolar partitions that often is followed by a diffusion intersticial sclerosis and signs of a pulmonary heart (see. Pulmonary heart). Increase in a liver, spleen, limf, nodes is noted. Can be observed a hypertrophy of gums, dedentition, focal thickenings and ulcerations of a mucous membrane went. - kish. path. Damage of skin is shown by papular rashes, hemorrhages, ulcerations are possible.
Microscopically tumorous granulematozny growths consist of histiocytes with impurity of the lymphocytes disseminated or lying in the form of accumulations, plasmocytes, eosinophilic granulocytes, hl. obr. segmentoyaderny. Siderophages can meet (see. Brown at plotneny lungs). Existence of a large number ksantomny (quite often huge multinuclear) cells macrophages with foamy vacuolated due to accumulation of cholesterol and cholesterol-esterov cytoplasm is characteristic (tsvetn. tab., Art. 368,
fig. 5 and 6). The ratio of cellular elements can vary that reflects different stages of process. Cytoplasm of histiocytes is characterized by a sudano-filiya, diffusion ShIK-polozhi-telnoy reaction (see CHIC reaction), high activity of hydrolases. At electronic microscopic examination in cytoplasm of histiocytes so-called little bodies of X — the extended inclusions connected with a membrane of cells similar to lizosomopodobny granules of cells of Langergans of an acanthceous layer of epidermis come to light. As a rule, there are centers of a necrosis, hemorrhage and in a late stage — fields of a sclerosis.
Fig. 1. Outward of the child with a disease Hend — Schueller — Krischena: a bilateral exophthalmos, damage of skin in the form of xanthomas on a front surface of a trunk.
Clinical picture. At damage of bones of a skull and a basin a frequent symptom of a disease — an ostealgia. At damage of a temporal bone noninfectious average otitis (see), steady against antibacterial therapy can develop. Owing to destruction of bones of an eye-socket there is an exophthalmos (fig. D), at destruction of the Turkish saddle dysfunction of a hypophysis, a hypothalamus with the phenomena of not diabetes mellitus is noted (see Diabetes not sugar). Destruction of bones of jaws promotes shaking of teeth, their loss, stripping of fangs, development of a secondary ulitis (see), stomatitis (see).
HENDA-ShYuLLERA-KRISChENA the DISEASE
of 493 U 50 — 60% of patients observes damages of skin in the form of the xanthomas painted in yellowy-brown color (see the Xanthoma), vesicles, ulcerations, hemorrhages. During the involvement in process of lungs cough, short wind are noted. The disease can have hron. the current and slowly to progress. Dissimination of process with defeat limf, nodes, a liver, a spleen and other bodies is possible.
In blood moderate anemia (fir-tree), a pancytopenia is sometimes noted (see. Hypoplastic anemia); in a marrowy iunktat — with
a hrannost of megacaryocytes, sometimes infiltration by histiocytic elements.
In rare instances complications are patol. changes. So, damage of a backbone can lead to a compression fracture of vertebras (see the Backbone), to cause a prelum of a spinal cord and radicular symptoms (see. Spinal cord).
The diagnosis is established on the basis by a wedge, pictures, results tsitol. and gistol. researches of the centers of defeat, and also rentgenol. researches of bones.
In an iunktata of tumoral cutifications and soft tissues reveal infiltration by atypical histiocytic cells with macrophagic function, signs of an erythrophagocytosis; in process of progressing of a disease such infiltrates find in marrow.
Rentgenol. the picture is characterized by preferential damage of bones of a skull (fig. 2), a basin, less often vertebras, edges and long tubular bones are involved in process (femoral, humeral, bones of a shin). In bones of a calvaria multiple defects of incorrectly rounded or oval shape with accurately outlined corroded contours without symptoms of osteoporosis and reactive sclerous changes on the periphery are formed. The sizes of the centers vary, sometimes the centers occupy extensive sites: bones. Destructive processes in the field of a pyramid, a mastoid and other departments of a temporal bone are the reason of damage of an ear. The Turkish saddle is, as a rule, not changed. In some cases its small consolidation comes to light. From bones of a facial skull of change are found in jaws, especially in upper more often. At defeat of alveolar shoots expansion of tooth sockets is observed. In the field of a basin the centers of destruction are localized preferential in wings of ileal bones. Damage of vertebras more often happens odi-
Ry. 2. The roentgenogram of a skull at Hend's disease — Schueller — Krischena: couples
a cue the outlined defect of a bone tissue in a frontal bone.
Fig. 3. The roentgenogram of a shin at Hend's disease — Schueller — Krischena: the extensive
site of destruction in a metadiaphysis of a tibial bone.
night, meets preferential in average and the bottom of chest department of a backbone. Sometimes the compression of bodies of vertebrae - with formation of a platibrakhispondi-liya develops (see the Backbone) at safe intervertebral disks. In rare instances patol. the fracture of a vertebra can be the first a wedge, a symptom of a disease. Tubular bones are blown up from within, contain the multiple centers of destruction of various size, cortical (compact) substance is thinned, sequesters are not observed. At subkortikalnokhm an arrangement of the centers there can be periostites (see).
At adults rentgenol. manifestations differ a little. More often long tubular bones are surprised, in to-rykh extensive periostoses develop (see Rickets). Destructive processes in bones of extremities (fig. 3) can be the cause them patol. changes. Changes in lungs are characterized more often by bilateral melkoochagovy shadows, and sometimes and a hyperplasia intrathoracic limf, nodes.
Differential diagnosis carry out with other forms of a histiocytosis of X, napr, with Letterer's disease — Siwa (see Letterer — Cuse a disease).
Treatment complex also depends on features of distribution of infiltrates. At focal damages of bones apply radiation therapy and an operative measure. The indication to operation is bystry increase in one or several centers (the centers in femoral, tibial, humeral, haunch bones, edges, vertebrae most often increase), considerable destruction of a bone, thinning of cortical substance, danger patol. a change, the accruing affected bone pains, emergence of contractures in a joint, lameness, a muscular atrophy.
The volume of an operative measure depends on localization of the center, its sizes, age and the general condition of the patient. Operation consists in a scraping or a resection of an affected area of a bone. As osteoplastic material for substitution of defect use an autokost or an allokost in the form of shaving, filling brick, cortical plates (see. Bone plastics). At minor regional defects the plastics is not made. Defects of the big size (in long tubular bones, nadatsetabulyar-ache areas of a basin) fill with cortical allotransplants. In tubular and flat bones the defects which are not exceeding 2 — 4 cm in the diameter carefully tampon an autostruzhka. At damage of a backbone reclination (see the Kyphosis) and fixing by means of metal brackets is shown. In bones of a calvaria the plastics is carried out plates from an allokost or from perforated plastic. In the postoperative period (see) the immobilization by a plaster bandage before reorganization or a reparation of a bone is shown. It is noted that operational treatment of one of the centers quite often leads to spontaneous closing of several centers in other bones. A recurrence after an operative measure usually is not celebrated.
At generalized defeats of bone system appoint antineoplastic means — Vincristinum, vinblastine, Cyclophosphanum (cyclophosphamide), Chlorbutinum (leukeranum), Prednisonum, Mercaptopurinum, a methotrexate; in some cases — their combinations.
Forecast in most cases favorable. It worsens at damage of lungs and accession of intercurrent diseases.
Prevention is not developed.
Bibliography: Carrhae I., etc. A lymph
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