LYMPHOGRANULOMATOSIS

From Big Medical Encyclopedia

LYMPHOGRANULOMATOSIS (lymphogranulomatosis; lat. lympha pure water, moisture + granulomatosis[es]; synonym: Hodzhkin's disease, malignant granuloma, chronic malignant lymphomatosis) — the malignant new growth of an adenoid tissue which is characterized by its granulematozny structure with existence of colossal cells of Berezovsky — Shternberg, damage of lymph nodes and internals.

For the first time L. it is described in 1832 by the English doctor Hodzhkin (Th. Hodgkin) which reported about seven cases, proceeding with fervescence, increase in a spleen and limf, nodes. The first gistol, researches L. were carried out in 1875 by I. I. Kutyrev. In 1890 S. Ya. Berezovsky suggested about the tumoral nature of this disease and noted existence at L. huge multinucleate cells. In 1897 Q. Paltauf and in 1898 Shternberg (To. Sternberg) in detail described morfol, a picture L.; Shternberg, as well as S. Ya. Berezovsky, found characteristic colossal cells in granulematozny fabric, to-rye further received the name of cells of Berezovsky — Shternberg. Nek-ry foreign authors call them sometimes Shternberg's cells — D. Reed, paying tribute to careful studying and the description of features gistol, structures of these cells, carried out in 1902 D. Reed.

The first wedge. classification of L. offered in 1865 A. Trusso, and the first morfol, classification appeared only in 1928, it was offered by Teyplan and Mittelbakh (Teiplan, Mittelbach). Since 1947 Jackson and Parker's classification was used morfol (N. of Jackson, F. Parker), in a cut the paragranuloma, Hodzhkin's granuloma and Hodzhkin's sarcoma were allocated. Radical radiation therapy of L. it was implemented in a wedge, practice V. Peters,

L. L. which proved a basic possibility of treatment occurs at any age, a thicket at men; rise in incidence is noted aged from 20 to 35 and 50 years are more senior. According to Greco (R. Greco) et al., to the USA (State of Connecticut) the frequency of L. for the periods from 1935 to 1944, since 1945 on 1954 and from 1955 to 1962 gradually increased and made respectively 13, 20 and 21 case on 1 million population a year. In Japan L. meets seldom and it is almost not observed at young age, in the countries of Latin America — it is found in children, in Finland and Japan more often — among city dwellers.

Etiology

Aetiology of L. it is not revealed. At the beginning of 20 century wrong idea of the tubercular nature of L. Obsuzhdayutsya of a problem of the virus and genetic theory of an origin of a disease, however, according to most of authors and according to the International classification of tumors of the hemopoietic and adenoid tissue (WHO, 1976), L dominated. is a version malignant lymphoma (see).

A pathogeny

In a pathogeny of L. the great value is attached immunol, to changes and first of all dysfunction of T lymphocytes that is expressed in suppression of cellular immunity, in high sensitivity of sick L. to virus (e.g., chicken pox) to both fungus infectious diseases and oppression of developmental potency of allergic reactions of hypersensitivity of the slowed-down type (skin reaction to tuberculine and antigens of a bacterial, virus and fungal origin; the slowed-down rejection of an allogenic skin transplant). Immunol, nek-ry authors explain with damage frequent development at L. hemolitic anemia, a leykotsitopeniya, a Werlhof's disease and a combination of H.p. diseases, in a pathogeny to-rykh many authors the leading role is given to autoimmune processes (a pseudorheumatism, nephrite, etc.). At nek-ry sick L. in an end-stage the picture reminding a so-called homologous disease can develop [the syndrome which is developing at introduction to animals and the person of allogenic lymphoid cells and characterized by development in adenoid tissue (see) processes of proliferation with the subsequent its exhaustion and an atrophy]. It is possible that at L. lymphoid cells at the expense of recurrent vegetative mutations can become alien and promote development patol, the process reminding so-called reaction «a transplant against the owner» (see. Incompatibility immunological ).

Pathological anatomy

Fig. 1. Macrodrug of sharply increased periaortal lymph nodes at a lymphogranulomatosis (the ventral aorta is opened).
Fig. 2. Macrodrug of sharply increased tracheal lymph nodes at a lymphogranulomatosis (the trachea is opened).

The first morfol, manifestation of L. usually increase cervical or supraclavicular limf, nodes is, less often primary center can arise in other groups peripheral and visceral limf, nodes, and also in nek-ry bodies (e.g., in lungs, went. - kish. path). Macroscopically struck limf, nodes are increased in sizes, condensed, grayish or gray-pink color on a section, sometimes soldered in hilly conglomerates (tsvetn. fig. 1 and 2).

In fabric limf, nodes the fibrous layers dividing it into separate small knots with noticeable, sometimes irregular shape yellowish sites of a necrosis quite often meet. The capsule limf, nodes is, as a rule, kept, its germination can be observed only in late stages of process, especially when limfogranulematozny fabric becomes indistinguishable from low-differentiated sarcomas (see) also infiltrirut surrounding fabrics and bodies. Morphological changes in a spleen at L. are found in 45 — 70% of cases.

Fig. 3. The macrodrug "porfirny" of a spleen at a lymphogranulomatosis: on a section the Centers of tumoral fabric are visible numerous, various size (are specified by shooters).

The mass of a spleen can increase till 1000 and more, however in most cases L. it does not exceed 500 g or remains normal. But also with a normal mass of a spleen in it by means of careful microscopic examination it is possible to find inherent L. focal changes. On a section of a spleen in typical cases the grayish tumoral small knots appearing on a dark red background and giving it the characteristic motley look which received classical definition «a porfirny spleen» for similarity with shlify a stone of porphyry are visible (tsvetn. fig. 3). In tissue of a spleen focal manifestations of L. are combined with a lymphoid hyperplasia (especially in initial stages) and a hemosiderosis because of hemorrhages and the strengthened hemolysis of erythrocytes.

Fig. 4. Macrodrug of a lung with the increased radical lymph nodes at a lymphogranulomatosis: germination of tumoral fabric on the surface of a visceral pleura (a part of pulmonary fabric is removed).

Macroscopically at L. lungs distinguish diffusion and infiltrative (mediastinal and radical), large and melkoochagovy and massive (sclerous and pnevmoniyepodobny) forms. Can be involved in process a pleura (tsvetn. fig. 4), large bronchial tubes, trachea; massive pnevmoniyepodobny L. can be followed by cavitation.

On morfol, to data, L. went. - kish. a path carries hl. obr. secondary character as a result of distribution of process with struck retroperitoneal and mesenteric limf, nodes. However in some cases primary defeat meets went. - kish. a path, at Krom tumoral growths at first are preferential on site group and solitary lymphoid follicles, and infiltration of muscular and mucous covers with the subsequent ulceration, bleeding, and sometimes with perforation of a wall and development of peritonitis is noted further.

At morfol, a research of a liver limfogranulematozny changes are found not always even at sharp (to 4 kg) increase in mass of body; distinguish small - macrofocal and diffusion defeat.

In a pathoanatomical picture L. kidneys changes in interstitial fabric with secondary atrophic and dystrophic changes in a parenchyma of body prevail.

Morfol. changes of a nervous system at L. develop most often on continuation upon transition of process from the affected bones of a skull, vertebrae or limf, nodes. Hematogenous metastasises of L. in a brain are found seldom.

Among changes of bones at L., on a nek-eye to data, St. 50% make damages of a backbone, in Krom single are defined, the multiple centers of destruction are more rare, is more often in spongy substance of bodies of vertebrae, sometimes in handles and shoots. On frequency damages of edges, then a breast and haunch bones are on the second place. L. tubular bones and bones of a skull it is observed less often.

At L. almonds, heart, mammary glands, a thyroid gland, eyes, ovaries, a uterus, testicles, serous covers, etc. can be also surprised, to-rye are involved in patol, process in an end-stage of a disease or at generalization of tumoral process.

Fig. 1. Microdrugs of a lymph node at a lymphogranulomatosis: and — Berezovsky's cell — Shternberg (it is specified by an arrow); — Hodzhkin's cells (are specified by shooters).

Microscopic picture L. it is characterized by growths lymphoid and plasmocytes, histiocytes, fibroblasts, eosinophilic and neutrophilic leukocytes, proliferation of cells of an endothelium of vessels that in total creates the motley picture reminding by the form a structure of granulyatsionny fabric. In some cases so-called xanthomatous L. cytoplasm of histiocytic elements contains inclusions of lipids. Against the background of a granulomatosis colossal cells of Berezovsky are found — Shternberg (fig. 1, a), the main distinctive morfol, features to-rykh are the huge size of cytoplasm and kernels, signs of nuclear fission without division of cytoplasm and the large kernels reminding intranuclear inclusions. Kernels of cells of Berezovsky — Shternberg large, to dia. 40 — 80 microns, with the gentle network of chromatin which is condensed to the periphery that creates the central area of an enlightenment in a kernel. One of options of cells of Berezovsky — Shternberg — so-called lacunary cells (fig. 3, c) with kernels of smaller size and plentiful, very accurately outlined, usually light, sometimes optically «empty» cytoplasm. Lacunary cells well come to light during the fixing of material in solution of formalin; use for fixing, e.g., does not result solutions of chromic salts in such effect. Berezovsky's cells — Shternberg at different morfol, types L. meet in various quantity, at the same time in an end-stage they differ in polymorphism and hyperchromic kernels. High specificity for L. Berezovsky's cells — Shternberg formed the basis calling them diagnostic cells. At L. also so-called cells of Hodzhkin (fig. 1,6) — the colossal one-nuclear cells which are, on a nek-eye to data, predecessors of cells of Berezovsky — Shternberg, but the nuclear fissions deprived of signs meet.

According to gistol, classification of L., offered in 1966 by R. J. Lukes et al., on a quantitative ratio of cellular elements and character of a sclerosis allocate four gistol, type L.: limfogistiotsitarny (lymphoid dominance), smeshanno-cellular, nodulyarny sclerosis and lymphoid exhaustion (reticular option and diffusion fibrosis). Authors of this classification established kliniko-morfol, parallels, on the Crimea for early stages and is long the current forms L. dominance of lymphocytes or nodulyarny character of a sclerosis are characteristic, and trace amount of lymphocytes is noted at a subacute current of L. and in a stage of generalization of process.

Fig. 2. Microdrug of a lymph node at limfogistiotsitarny type of a lymphogranulomatosis: proliferation of lymphocytes is visible, the arrow specified Berezovsky's cell — Shternberg.

Limfogistiotsitarny type L. it is characterized by dominance of the lymphoid proliferation which is combined with various extent of proliferation of histiocytes. Proliferation of cells can have focal or diffusion character, eosinophils and plasmocytes are located unevenly, colossal cells of Berezovsky — Shternberg meet seldom; usually there are no centers of a necrosis (fig. 2).

At smeshanno-cellular type L. the motley cellular composition of fabric with a large number of cells of Berezovsky — Shternberg and existence of the centers of a necrosis is marked out. Process quite often takes all fabric limf, a node, but also focal defeats meet. Sometimes at this type L. small growths of reticular and collagenic fibers are found.

Fig. 3. Microdrugs of a lymph node at a lymphogranulomatosis with a nodulyarny sclerosis: and — the node of tumoral fabric surrounded with ring-shaped growths of collagenic fibers is visible (it is specified by an arrow); — collagenic fibers, impregnirovanny caustic silver are visible (are specified by an arrow); in — lacunary cells of Berezovsky — Shternberg (are specified by shooters).

The Nodulyarny sclerosis is characterized by development of the peculiar coarse-fibered ring-shaped layers of connecting fabric dividing fabric, e.g. limf, nodes on separate small knots (fig. 3, and, b) with the cellular structure corresponding to limfogistiotsitarny and smeshanno-cellular types, and also reticular option L. Morfol, feature of a nodulyarny sclerosis frequent dominance in tumoral fabric of lacunary cells of Berezovsky — Shternberg is (fig. 3, c).

Fig. 4. Microdrug of a lymph node at a lymphogranulomatosis with exhaustion of an adenoid tissue: tissue of a lymph node is poor in cellular elements, with fields of a diffusion sclerosis.
Fig. 5. Microdrug of a lymph node at reticular option of a lymphogranulomatosis: numerous polymorphic reticular macrophages are visible (are specified by shooters).

The expressed chaotic development of connecting fabric and reduction of total number of cells first of all at the expense of lymphocytes (fig. 4) is characteristic of lymphoid exhaustion at diffusion fibrosis. The reticular option is characterized by the expressed proliferation of atypical histiocytes (fig. 5) and Berezovsky's cells — Shternberg.

Described morfol, types are not the stiffened forms L. Relative stability morfol, composition of fabric, according to most of authors, most longer remains at a nodulyarny sclerosis. During the progressing of L. the accruing changes lead to transformation gistol, types from limfogistiotsitarny to lymphoid exhaustion, a cut, as a rule, prevails in morfol, a picture at a research of autopsiyny material.

One of frequent complications of L. — amyloidosis (see), to-ry meets, according to various data, in 8 — 12% of pathoanatomical openings. From associated diseases at L., on a nek-eye to data, in 15% of cases fibronodular tuberculosis, usually without signs of progressing is found.

The clinical picture

the Clinical picture is characterized by dynamism of a current of L., variety of symptoms and forms * various bodies and groups caused by defeat limf, nodes.

Acute L. proceeds with high temperature, increase limf. nodes, the spleen and a liver expressed by anemia it is frequent with positive reaction of Paul — Bunnellya (see. Paul — Bunnellya reaction ). Optional version of acute L., shown only high temperature in combination with an adynamy, weight loss, anorexia, abdominal pains, anemia and sharply accelerated ROE. Transitional on character a wedge, currents of a form were considered as subacute L earlier. The current of H.p. by limited defeat of one group limf, nodes is in most cases observed hron. At generalization, edges most often happens gradual, symptoms of the general intoxication amplify, bodies and systems are involved in process.

On international a wedge, the classification accepted in 1965 and modified in 1971 is allocated by four stages of prevalence of L.: The I stage (local forms) — defeat of one or two adjacent groups limf, the nodes located on one side of a diaphragm; The II stage (regional forms) — defeat of two or more non-adjacent groups limf, the nodes located on one side of a diaphragm; The III stage (generalized forms) — defeat on both sides of a diaphragm and involvement in process of a spleen; The IV stage (the disseminated forms) — defeat limf, nodes and internals — lungs, a liver, kidneys, etc.

Each stage is subdivided, in turn, depending on absence (A) or existence (B) of one or several symptoms of intoxication: fervescences are higher 38 °, night sweats, a skin itch, a bystry lose of weight more than for 10% (at the same time existence only of a skin itch is considered insufficient for reference of a case to subgroup of B).

Except the general symptoms of L., are considered also so-called biol, signs of activity of tumoral process, to the Crimea belong the accelerated ROE, a neutrophylic leukocytosis, a high level of fibrinogen in blood, low content of serumal iron, giper-alfa2-and hypo - gamma globulinemiya, etc. (absence or existence of signs biol, activities designate the small letters «and» and «b»).

Except a wedge, stages, stages can be established so-called patol., in to-rykh more precisely, g extent of distribution of process is defined by the help of the data obtained at a laparotomy. The diagnostic laparotomy with a biopsy of internals and limf, nodes allows at I and II wedges, stages to reveal other centers of L. in an abdominal cavity and to remove a spleen as a possible source of dissimination of process. Patol, stages designate by means of the special symbols showing existence or lack of changes in taken for gistol, researches during a laparotomy limf, the N+ or N nodes — (Nodus); a liver of H+ or N — (Hepar); spleens of S+ or S — (Spleen); easy for L + or L — (Lung); marrow of M+ or M — (Marrow), a pleura of P+ or P — (Pleural); bones of O+ or O — (Osseus); skin of D + or D — (Derma).

Fig. 6. Type of patients with a lymphogranulomatosis: about — with damage of subclavial lymph nodes, a lymphostasis in the left hand and chest gland; — with a face edema and a shoulder girdle on the right at a compression syndrome of an upper vena cava, on skin traces are visible having combed.

Wedge, picture L. in the I stage it is shown by the only constant symptom — increase in one or several peripheral limf, nodes of one anatomic area (fig. 6, a). The general condition of patients in the I stage usually does not change, however in blood, according to nek-ry authors, the neutrophylic leukocytosis with band shift, a lymphocytopenia and the accelerated ROE is found in a number of patients; the tendency to hypo - gamma globulinemii is noted.

In the II stage in most cases the earliest symptom is increase peripheral limf, the nodes located above a diaphragm. As well as in the I stage, the neutrophylic leukocytosis meets shift to the left, the lymphocytopenia, is slightly more often noted the accelerated ROE; there is more expressed tendency to hypo - gamma globulinemii and a clear fibrinosis.

In PB stages, besides, are noted the general symptoms of a disease — weakness, perspiration, high temperature, a skin itch, the dry cough caused by defeat limf, nodes of a mediastinum.

In IIIA of a stage the course of a disease differs in relative high quality. According to many authors, approximately at 70% of patients the long time is not broken the general state and working capacity. As well as in IIA of a stage, complaints to moderate perspiration, temperature increase to subfebrile figures, increase peripheral limf, nodes and emergence of the pains caused by this increase are noted. In IIIA stages considerably meet complaints to a skin itch more often.

In IIIB of a stage the disease proceeds hard: almost all patients show complaints to weakness, an indisposition, high temperature, perspiration, a skin itch, a big lose of weight and some other symptoms is often noted.

In IIIA and IIIB stages approximately with an identical frequency are found a neutrophylic leukocytosis with shift of a formula to the left, the lymphocytopenia accelerated by ROE, and also a fibrinosis and hyper-alfa2-globulinemiya.

Damage of internals (the IV stage) is most often observed at generalization of L., but can also develop as a result of local distribution of tumoral process from regional limf, nodes on departments of parenchymatous bodies, adjacent to them. Very seldom, according to Wood and Keltmen (N. Wood, Ch. Celtman), approximately in 0,25% of cases, is noted primary damage of internals.

Complex combination wedge, manifestations of L., caused by features of localization of tumoral process, existence or lack of the general symptoms, various speed of progressing of a disease, frequent accession nonspecific or so-called. paraneoplastic syndromes (see), allows to allocate a skin, bone, neurologic, thoracic wedge, forms, and also forms with the preferential or isolated defeat went. - kish. path, etc.

Fig. 7. The crateriform ulcer with an uneven nekrotizirovanny bottom at a lymphogranulomatosis of skin and soft tissues.

Primary L. skin its wedge is among extremely rare, forms. Isolated cases of a nodulyarny sclerosis with the multiple isolated damage of skin are described. Secondary changes of skin at L. meet in 10 — 20% of cases. At the same time specific manifestations arise both in initial, and in an end-stage of a disease. They can be single and multiple, in the form of the separate small knots with a diameter from several millimeters to 3 — 5 cm or extensive infiltrates of usually rounded shape of dark red color surrounded with quite often pigmented and edematous skin. Their most frequent localization — area of a back, top and bottom extremities. Secondary damages of skin can be caused by germination in it tumoral fabric from limf, nodes, a mammary gland, a breast, edges with the subsequent formation of ulcers up to 10 cm in the diameter and more with plentiful is purulent - necrotic separated (fig. 7).

At L. often, especially in end-stages of a disease, nonspecific toksiko-allergic changes of skin, an origin to-rykh finally are observed it is not found out. According to one authors, they are caused by anaphylactic reactions of an organism, others consider them the investigation of irritation of a sympathetic part of century and. page toxicants. Rashes like eczema, urticarias, an itch of skin belong to nonspecific damages of skin, to bark - and scarlatiniform rashes, an erythema, a hyperpegmentation of skin, to-rye can be localized and generalized. The skin itch and rashes like a prurigo in the form of small firm papules of pink color with excoriations are most often observed. The current of a basic disease quite often burdens shingles.

Damage of bones at L. develops most often for the second time, usually in those departments of a skeleton, to the Crimea close prilezhat big groups limf, nodes. Bone changes arise preferential against the background of generalization of process, sometimes they are the only manifestations of L.

Conducting a wedge, symptoms at L. vertebrae the pains localized or irradiating are to-rye are explained by a prelum of nervous trunks or germination in them tumoral fabric. Pains can amplify during the pressing on acanthas of the affected vertebras; at localization of process in nizhnegrudny and verkhnepoyasnichny vertebrae they irradiate in the lower extremities; there are a feeling of numbness, weakness and twitching in legs. Prelums of a firm meninx of a spinal cord can be caused paresis and paralyzes of the lower extremities with dysfunction of bodies of a small pelvis.

Constant symptoms during the involvement in process of edges and a breast are pains and emergence of myagkotkanny tumorous education according to an affected area; at a palpation of a breast the characteristic sound — a symptom of a so-called pergament crunch sometimes is defined.

Changes in pelvic bones are connected with germination in them tumoral fabric from ileal and pelvic limf, and are much more rare than nodes as a result of hematogenous distribution of process. The pains of constant or pristupoobrazny character sometimes irradiating in legs that is explained by pressure of tumoral masses upon neuroplexes are characteristic of this localization.

Wedge, a picture of defeat of pulmonary fabric is defined by prevalence of process, existence of symptoms of intoxication and complications; thorax pains, cough, short wind, sometimes a pneumorrhagia are noted.

Primary L. mediastinums it is usually characterized by a long asymptomatic current. At the same time increase mediastinal limf, nodes long time can remain the only manifestation of process. Subjective manifestations in most cases arise in connection with development of symptoms of a prelum of bodies of a mediastinum.

Further at local progressing of process signs of defeat of large vessels in system of an upper vena cava with a typical picture of a mediastinal compression syndrome (fig. 6, b), symptoms of defeat phrenic and returnable guttural nerves, exudative pleurisy, a pericardis, etc. can develop.

At L. went. - kish. a path the small bowel is surprised more often, the stomach and a gullet are more rare; L. a large intestine meets seldom. Wedge, picture L. went. - kish. a path it is caused by local and general symptoms of a disease, considerably depends on the nature of growth of a tumor, the sizes and localization patol, the center. To establish precursory symptoms of defeat went. - kish. a path difficult since it usually arises when there are expressed general symptoms of a disease. L. a stomach and a duodenum sometimes proceeds about a wedge, the symptoms inherent to a peptic ulcer: nausea, pains in epigastriß area and the right hypochondrium, morbidity are noted at a palpation of a stomach. For L. a small bowel abdominal pains, a meteorism and diarrhea are characteristic.

Primary damage of a spleen meets seldom and is characterized by relative high quality of a current — cases when process during a long span remains localized are known that the wedge, forms L is the basis for allocation by nek-ry authors so-called splenic. At damage of a spleen there can be pains in left hypochondrium, amplifying in situation on the left side, during the walking, run.

Diagnosis

Diagnosis of L. is based on the analysis of clinical, radiological and morphological data. The major role in recognition of L. belongs morfol, to methods of a research — to a cytologic research of punctate limf, a node, allowing to establish the preliminary diagnosis, and to a histologic research of the material received with the help biopsies (see), including and at diagnostic laparotomies (see). Usually both of these methods are used consistently and supplement each other. Diagnosis of L. on gistol, to drug it is possible only at detection in the studied fabric of colossal cells of Berezovsky — Shternberg. Existence in drugs of cells of Hodzhkin allows to state only the presumable diagnosis, to-ry is not the basis for performing pathogenetic treatment.

Special difficulties for a differential morfol, the diagnosis represent limfogistiotsitarny type and diffusion fibrosis. Limfogistiotsitarny type L. it is necessary to differentiate with a reactive hyperplasia of an adenoid tissue of various etiology (infectious, virus, postvaccinal, medicinal, etc.), specific lymphadenites at nek-ry inf. diseases, napr, brucellosis (see) and toxoplasmosis (see), and also with sarcoidosis (see) and lymphocytic lymphosarcoma (see). At lymphoid exhaustion as diffusion fibrosis it is necessary to exclude fibrosis of fabric limf, a node of an inflammatory origin, and at reticular option L. it is necessary to carry out differential morfol, the diagnosis with a lymphosarcoma and reticulosarcoma (see).

For the wedge, diagnoses of L is more whole. searches a lab are conducted. the signs reflecting activity of process, napr, the content in blood of ceruloplasmin (see. Respiratory pigments ), gaptoglobina (see), and also alkaline phosphatases (see), a hexosemine (see. Aminosugar ), etc.

In 1971 on a symposium to Ann Arbor (USA) the system a wedge, diagnoses of L was offered., on a cut obligatory researches are detailed studying of the anamnesis of a disease and a wedge, symptoms, a biopsy of one of struck limf, nodes (it is desirable not inguinal), a lab. methods (bulk analysis of blood, determination of level of an alkaline phosphatase in blood serum), a research of function of kidneys and a liver, a X-ray analysis of bodies of a thorax and bones, an intravenous piyelografiya, a bilateral lower X-ray contrast limfografiya.

Under certain conditions conduct additional researches — a tomography of bodies of a thorax (see. Tomography ), lower kavografiya (see), trepanobiopsiya (see), a laparotomy with a splenectomy (at suspicion on damage of abdominal organs).

From among auxiliary researches apply skeletal stsintigrafiya (see), scanning of a liver and spleen (see. Liver, radio isotope research ; A spleen, a X-ray radiological research), nek-ry biochemical, blood analyses, napr, determination of content in blood serum of calcium and uric to - you, definition of developmental potency of allergic reaction of hypersensitivity of the slowed-down type.

Fig. 8. The roentgenogram of a thorax (a direct projection) at a lymphogranulomatosis: moderate expansion of upper parts of a median shadow is defined, it is more to the left (it is specified by an arrow).
Fig. 9. The direct tomogram of a thorax at a lymphogranulomatosis of lungs: in a radical zone roundish shadows of various sizes and density are visible at the left (are specified by shooters).

At rentgenol, a research of bodies of a thorax at L. the expanded median shadow (fig. 8), a thicket in upper and average departments caused by increase tracheal, tracheobronchial and bronkhopulmonalny limf, nodes can be defined. Depending on defeat of various groups limf, nodes the median shadow can be symmetric or asymmetric.

At a diffusion and infiltrative form L. lungs the illegibility of contours on site of germination of a node in tissue of a lung is defined, the radiarny tyazhistost, edges is lost in process of removal from a root. Focal forms L. lungs have usually homogeneous structure of blackout: in one cases the centers are accurately outlined and remind metastasises of a tumor, in others — have an appearance of spotty shadows with indistinct contours (fig. 9). At a melkoochagovy form L. lungs the large number of small various form and density of the isolated centers radiological is defined, to-rye are located in one or is symmetric in both pulmonary fields and give the picture similar to a carcinomatosis of lungs.

Fig. 10. A direct limfogramma of retroperitoneal space and a small pelvis at a lymphogranulomatosis: contrast shadows of the increased lymph nodes (are specified by an arrow) with heterogeneous» the coarse-grained drawing are visible.

Diagnosis, scoping and extents of defeat retroperitoneal limf, nodes are carried out by means of an angiography and limfografiya (see), especially in combination with flebografiya (see). On limfogramma most often it is possible to find increase in shadows limf, nodes (fig. 10) with the kept equal, accurate contours, and also structural change of nodes, edges becomes heterogeneous, coarse-grained, sometimes «foamy». In bringing limf, vessels narrowing of gleams and formation of collaterals is noted.

Fig. 11. The roentgenogram of an abdominal cavity with contrasting of intestines (a direct projection) at a lymphogranulomatosis: multiple minor semi-roundish defects of filling (are specified by shooters) with uneven contours are visible.

At L. went. - kish. a path with an endophytic form of defeat irregularity of filling and passing of a contrast suspension, change of a form of a gleam or deformation of the struck body, rigidity of walls, a smoothness of a relief of a mucous membrane are observed. Less often defect of filling or defect on a contour, and also change of a relief of a mucous membrane come to light. At exophytic forms defect of filling is defined more often, disturbance of passability and deformation of body meet hl. obr. at tumors of the big sizes (fig. 11). In initial stages of L. a small bowel uneven advance and balling up of a contrast suspension, existence spastic of the reduced segments and defects of filling, disappearance characteristic, e.g., for a jejunum of a so-called plumose relief of a mucous membrane radiological are defined. In process of progressing of process the further thickening of folds with formation of subauriculate protrusions is observed, the gleam of a gut is narrowed, its walls become rigid, advance of a contrast suspension is slowed down. In an abdominal cavity are applied to specification of prevalence of process a mezenterikografiya, splenoportografiya (see) and tseliakografiya (see). On angiograms at the same time regional defects of filling, a prelum and shift of vessels, their Suprastenotichesky expansion, development of collaterals and other changes which are indirectly indicating increase limf, nodes come to light.

At rentgenol, a research of a skeleton structural changes of a bone tissue are defined, to-rye are characterized by a combination of osteoplastic productive processes. Osteoklastichesky changes can have an appearance of the solitary centers with indistinct and uneven contours or enough accurately outlined centers with a narrow sclerous rim. Sometimes at L. multiple melkoochagovy destruction of a bone is observed, the affected area a cut takes a characteristic porous form. Periosteal reactions are absent more often, however at L. separate cases of periostoses of toxic character like Mari — Bambergera are described (see. Bambergera-Mari periostosis ).

Treatment

For treatment of L. apply radiation therapy, various antineoplastic cytostatic means and regarding cases — operational methods. The choice of the leading method of treatment is defined, first of all, by a stage of a disease: at I, by II and IIIA stages preference is given to radical radiation therapy, and at IIIB and the IV stages — chemotherapy.

Radiation therapy of L. it is based on use biol, actions ionizing radiation (see). It is applied palliative (local) and radical radiation therapy (see). At local radiation therapy radiation of the revealed center of defeat with the palliative purpose is carried out; radical radiation therapy is directed, besides, and to the fields of probable distribution patol, process, pursues the aim of radical impact on all centers of L. to resistant a wedge, recovery.

Radiation therapy of L. it is contraindicated at the expressed symptoms of intoxication, anemia, a leukopenia, thrombocytopenia, a cachexia, active forms of tuberculosis and beam injuries of skin and hypodermic cellulose caused by the radiation which was carried out earlier.

Radiation therapy of L. the hl is carried out. obr. by means of gamma and therapeutic devices (see. Gamma devices ), in to-rykh a source of radiation the radioactive nuclide serves 60 Co, and electronic accelerators.

Individual planning of radiation is developed taking into account a stage and morfol, type L., age and sex of the patient, localization of the centers of defeat, speed of progressing of process and existence of associated diseases. The choice of fields of radiation, their sizes, a form and localization are established on the basis of data rentgenol, and angiographic researches.

Fig. 12. The scheme of options of radiation at radical radiation therapy of a lymphogranulomatosis: 1 — at multifield radiation; 2 — at krupnopolny radiation (the sites which are exposed to radiation are shaded); and — an anterior aspect — the back view.

Radical radiation therapy of the localized forms L. it is carried out in two technical options — multifield and krupnopolny (fig. 12). The multifield option of radiation therapy provides consecutive radiation of sites of defeat and zones of possible distribution of process. At the same time the distance between adjacent fields shall correspond to geometrical borders of bunches so that the next bunches (above - and underlying fields) crossed at the level of a target. At radiation limf, nodes of a mediastinum and retroperitoneal space from back fields the spinal cord is shielded by lead blocks to the II lumbar vertebra.

The Krupnopolny option of radiation is carried out from two opposite mantiyevidny fields — front and back — with shielding by individually picked up figured blocks of lungs, heads of humeral bones, throats (at radiation in front) and a spinal cord (at radiation behind). The upper mantiyevidny field includes everything naddiafragmalno located limf, nodes. At radiation in front — the upper bound of the field of radiation is projected at the level of edge of a mandible at the head which is thrown back back, lower — at the level of a xiphoidal shoot; at radiation behind the lower bound of the field of radiation usually is at the X—XI level of chest vertebrae. At krupnopolny radiation danger of emergence of so-called hot and cold zones on border of the neighboring fields is excluded, duration of a course of beam treatment is reduced.

Limf, the nodes located below a diaphragm irradiate usually from two large opposite fields, shaped, the reminding turned letter «at», however krupnopolny radiation is hard transferred by patients because of quickly arising dispeptic disorders and oppression of a blood formation. Therefore for the purpose of radiation subphrenic limf, nodes usually use multifield radiation in two stages: at the first stage are affected by ionizing radiation retroperitoneal limf, nodes and a spleen, at the second stage — ileal, inguinal and femoral limf. nodes.

At IA and IIA a wedge, stages of L. nek-ry authors recommend to subject to radiation all main departments limf, systems; according to other authors, in these stages of process radiation of L, adjacent to the centers, is enough. areas limf, systems.

At IB,IIB and IIIA stages radiation therapy of all departments limf, systems on both sides of a diaphragm is carried out (to IIIB of a stage — in combination with chemotherapy). Total absorbed dose of radiation at limfogistiotsitarny type L. and a nodulyarny sclerosis in the centers of defeat shall make not less than 4000 I am glad for 4 — 4,5 weeks of treatment, and in zones of preventive radiation — 3000 — 3500 I am glad. At smeshanno-cellular type and reticular option L. the total absorbed dose increases on average for 1000 is glad that is caused adverse a wedge, the course of a disease at these morfol, types of H.p. by frequent recuring and a tendency to generalization.

At radiation therapy of L. there can be various beam damages (see), to-rye force to interrupt sometimes a course of treatment, oversetting the plan of radiation. Most often changes of the hemopoietic fabric with a picture of a leykotsitopeniya develop, degree of manifestation a cut depends on the volume of tissue of the marrow getting to the radiation zone. After radiation limf, the nodes located above a diaphragm there comes the moderate leykotsitopeniya due to small reduction of number of neutrophils and more expressed decrease in number of lymphocytes. At radiation limf, the nodes located on both sides from a diaphragm almost at all patients the maintenance of leukocytes falls lower than 3000 in 1 mkl blood. In nek-ry cases along with a leykotsitopeniya thrombocytopenia develops, the edge, as a rule, is not followed by hemorrhagic manifestations.

Perikardita, late pneumonites, indurative hypostasis and fibrosis of skin and hypodermic cellulose, a hypothyroidism are among late beam complications. Rare complications are the postbeam cross myelitis, arteritis of vessels of a brain and kidneys, a beam epithelitis of an oral cavity, etc.

The main indications to use of chemotherapy as the leading method of treatment of L. IIIB and the IV stages of a disease, and also such heavy manifestations of L are., as a prelum of an upper vena cava, a spinal cord, the general bilious channel, a plentiful exudate in a cavity of a pericardium, etc. Tumoral fabric at L. has high sensitivity to many antineoplastic means, at a wedge, use to-rykh nek-ry selective features of action are considered: e.g., Natulanum (see) it is active at all localizations of process, but especially at L. went. - kish. path; vinblastine (see), on a nek-eye to data, is more effective at L. lungs; Cyclophosphanum (see) — at defeat of serous covers and soft tissues; Bruneomycinum (see) — at damage of lungs, bones and a liver; Natulanum and nitrozometilmochevina (see) — at the expressed symptoms of intoxication, etc.

Results of treatment of H.p. the help of antineoplastic means improved owing to prolonged use of the cyclic polychemotherapy based on a combination of the drugs which are belonging to the different classes of connections and differing on the mechanism of cytostatic action. According to De Vita (V. T. De Vita) and soavt., the polychemotherapy according to the scheme MORR [Embichinum (Mustargenum), Vincristinum (Oncovinum), Natulanum (Procarbazinum) and Prednisolonum] leads to remission at 60 — 80% of patients with generalized L. Treatment according to the scheme MORR is carried out by two-week cycles (6 bucketed cycles in 2 weeks between cycles, at the same time only the first and fourth cycles include Prednisolonum).

The monochemotherapy as an independent method of treatment is used only in hard cases of a disease with the symptomatic purpose or applied as the so-called supporting treatment at patients with full remissions of L., received as a result of radical radiation therapy or poly-chemotherapy.

Many authors the program of treatment of L is recognized as the most rational. I, II and the III wedge, stages, including a diagnostic laparotomy with a splenectomy and the complex therapy providing the following three stages: the first stage — 2 — 6 cycles of polychemotherapy, the second stage — radical radiation therapy, the third stage — the supporting chemotherapy.

Operational treatment is used regarding cases at local forms of H.p. by the purpose of removal of primary L. a stomach and intestines with the subsequent preventive beam and chemotherapy. Operational treatment of L. it is carried out also according to vital indications, napr, at perforation or bleeding from specific ulcers went. - kish. path.

At a sick local form L. at approach of pregnancy the decision on its preservation can be made; in other cases concealing big danger of generalization of process abortion is shown (except cases the person psikhol, a mood of the patient on preservation of pregnancy). Radiation before childbirth at epiphrenic localization of process can be only local, at subphrenic localization of L. radiation therapy during pregnancy is contraindicated. In cases of the expressed intoxication during pregnancy treatment can be appointed antineoplastic drugs. The lactation is after the delivery excluded. The children who were born at the women suffering from L., according to many authors, have no bent to development onkol, diseases.

The forecast

the Forecast depends from a wedge, stages, presence of the general and biol, signs of activity of process, morfol, like

L. S the help of radical radiation therapy, according to Kaplan and Rosenberg (H. S. Kaplan, S. Rosenberg), and also other authors, treatment of the localized forms L. it is possible to reach more than at 80% of patients, and use of polychemotherapy in cases of generalized L. is followed by achievement of full remissions at 80% of patients, from them at 60 — 70% they remain St. 10 years.

Many authors consider adverse in the predictive relation such morfol, types as lymphoid exhaustion and smeshanno-cellular type, and note beneficial effect on results of treatment and the forecast splenectomy (see).

Working capacity of sick L. depends on a stage of a disease, efficiency of the carried-out therapy, age, etc. According to a number of authors, in I, II and IIIA stages of L. at radical treatment apprx. 70% of patients keep working capacity.

The lethal outcome comes during generalization of L. The cachexia, anemia, intoxication, a pulmonary heart are its proximate cause, is more rare — inf. and purulent complications, liver failure and amyloidosis.

The lymphogranulomatosis at children

the Lymphogranulomatosis at children makes 12 — 15% of all malignant new growths at children's age. More often it is found at the age of 5 — 8 years. According to a number of authors, the big frequency of a disease at boys is noted, other authors of such feature do not note. From morfol, types the hl is found in children. obr. the limfogistiotsitarny type, meets a nodulyarny sclerosis less often. The wedge, picture is in most cases characterized by the acute beginning of a disease with increase limf, nodes in a neck and a mediastinum; to more rare initial manifestations of L. defeats supraclavicular, axillary, inguinal and others limf, nodes and internals belong. Defeat retroperitoneal limf, nodes quite often proceeds with the expressed pain syndrome, to-ry can be the cause for a wrong operative measure. Unlike adults, at children at L. symptoms of intoxication (a skin itch, high temperature of a body, profuse night sweats) are not characteristic, the eosinophilia is less often observed, however children, as a rule, have a generalization of process quicker that nek-ry authors explain anatomo-fiziol. features of an adenoid tissue at children's age.

Treatment of L. at children complex. In I and II stages of a disease radical radiation therapy is carried out, in III and IV stages the leading method of treatment is the chemotherapy according to the same schemes, to-rye are accepted at treatment of L. at adults, but in the corresponding doses.

At radical radiation therapy of local forms L., on a nek-eye to predictive data, in most cases on full remission with life expectancy at 70% of patients goes more than five years.



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I. A. Pereslegin, A. E. Vermel, E. M. Filkova; N. N. Pokrovskaya, G. A. Frank (stalemate. An.).

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