From Big Medical Encyclopedia

SPLENOMEGALY (Greek splen a spleen + megas, megaion big) — increase in a spleen as a result of its involvement in patol. process. The page is observed at many diseases.

In classifications W. Dameshek, constructed by the pathophysiological principle, the forms C. connected with proliferation of lymphoid and reticuloendothelial fabrics, with congestive, hemolitic processes, a myeloid metaplasia, and the mixed forms are allocated. On an etiopatogenetichesky sign W. Dameshek allocated infectious, granulematozny, neoplastic S., and also S. connected with portal hypertensia, disturbance of cellular and lipidic exchange and hemolitic. Boudler (A. Y. Bowdler, 1983) systematized S., having studied the kliniko-nosological forms of diseases which are followed by increase in a spleen. It allocated the following forms: Page at acute and hron. infections, napr, viral hepatitis, septicaemia, Cytomegaloviral infection, toxoplasmosis, syphilis; Page at inflammatory and granulematozny processes, napr, a serum disease, a berylliosis, Felti's syndrome; congestive S., napr, at the vnutripeche-night block, obstruction of a portal, splenic or hepatic vein; Page at hematologic diseases, napr, hemolitic anemias, myeloproliferative diseases; Page at tumors, napr, leukoses, a lymphosarcoma, an angiosarcoma, cancer, a melanoma; Page at diseases of accumulation, napr, diseases to Gosha, a histiocytosis; Page at other diseases, napr, at primary amyloidosis, a thyrotoxicosis.

Reasons, the causing S., are diverse. Carry to their number: the general disturbances of blood circulation (cirrhosis of Peak at chronic cardial compression) and local (portal hypertensia); bacterial acute infections (tifo-paratyphoid diseases, sepsis, pair tuberculosis, especially its tifoidny form is lovely) and chronic (a brucellosis, tuberculosis of a spleen, syphilis); viral infections (measles, clumsy rubella, acute viral hepatitis, inf. mononucleosis, inf. lymphocytosis, etc.); protozoan (malaria, toxoplasmosis, leushmaniosis, trypanosomiasis); mycoses (histoplasmosis, zymonematosis); helminthoses (schistosomatosis, echinococcosis, etc.); tezaurismoza (a disease to Gosha, Nimann's disease — Peak, Krischen's disease — Henda — Schueller, Letterer's disease — Siwa, an essential lipidemia, a glycogenosis, hemochromatosis, hepatocerebral dystrophy, primary amyloidosis); general diseases of bodies of a hemopoiesis (acute and hron. leukoses, trombotsitemiya, myelofibrosis, malignant lymphoma, multiple myeloma); hemolitic and sideroblastny anemias, pernicious anemia, hemoglobinopathies; splenogenny neutropenia, cyclic agranulotsptoz, immune Werlhof's disease and immune trombotichesky Werlhof's disease; autoimmune diseases (hron. hepatitis in a stage of an aggravation), including collagenic diseases (a system lupus erythematosus, a nodular periarteritis, a pseudorheumatism); benign and malignant tumors of a spleen, and also cyst, abscesses, heart attacks of a spleen.

In a basis pathogeny The page can be a hyperplasia of a spleen (primary and secondary); stagnation in a spleen, napr, at the intra hepatic or extrahepatic block; a delay of erythrocytes as a result of defects of erythrocytes or a spleen; a myeloid metaplasia, malignant cellular proliferation, accumulation of nek-ry metabolic products (see. Histiocytoses ); granulematozny and inflammatory processes, benign tumors, etc.

Identification of the increased spleen by means of a palpation most often does not represent difficulties. In some cases, when these palpations are not convincing, napr, if necessary to differentiate S. with the increased or dihundred-pyrobathing kidney or a retroperitoneal tumor, the survey X-ray analysis of an abdominal cavity is recommended, at a cut characteristic contours of a spleen are usually accurately distinguishable, or irrigoskopiya (see) — at S. the lowered splenic corner of a colon is often visible, and at a retroperitoneal tumor it is raised or normally located. In doubtful cases are shown also piyelografiya (see) or pneumoperitoneum (see). The most reliable are radio isotope (stsintigrafiya) and X-ray contrast methods of a research — a splenoportografiya (see), tseliakografiya (see), allowing to define a form, the sizes and an arrangement of a spleen. Trial is in rare instances shown laparotomy (see).

In a basis diagnoses The page lies recognition of the diseases causing its development. Anyway in the presence of S. are necessary bulk analysis of blood (see. Gemogramma ), research of punctate of marrow, serum proteins, and also carrying out functional trials of a liver and serol. researches on presence of autoantibodies.

The page quite often is followed by a gpner-splenizm (see. Spleen ), a cytopenia in blood and, as a rule, increase in quantity of cellular elements in marrow that is more often observed at portal hypertensia (see), diseases to Gosha (see. to Gosha disease ), Nimann's diseases — Peak (see. Nimanna — Peak a disease ), sarcoidosis (see), malaria (see), brucellosis (see), tuberculosis of a spleen, schistosomatosis (see), leushmaniosis (see), Felti's syndrome (see. Felti syndrome ). The mechanism of a hypersplenism can be explained with humoral braking of a marrowy hemopoiesis (perhaps, in connection with decrease in products of hematinic principles) patholologically the changed spleen, deposition of uniform elements of blood (sequestration) in a spleen, strengthening of a cytolysis in the conditions of the broken blood-groove or increase in phagocytal activity of macrophages of a spleen. The doctrine about the cooperated immune response on an antigen challenge of immunocompetent cells (see), and also their roles in development of autoimmune (autoagressivny) reactions allows a number of researchers to consider a spleen as body, in Krom products of antibodies, including autoimmune are carried out and to put forward the concept about the autoimmune nature of a cytopenia at a hypersplenism. Its confirmation is the positive effect from splenectomy (see), observed at a number of an etiopatogeneticheska of the heterogeneous diseases which are followed by an autoimmune cytopenia.

Splenomegaly at children meets more often than at adults. Partly it is connected with the fact that a number of the diseases which are followed by increase in a spleen, napr rickets (see), meets preferential at children's age. The spleen is, as a rule, increased at a disease to Gosha (see. to Gosha disease ), Nimann's diseases — Peak (see Nimann — Peak a disease), Letterer's diseases — Siwa (see. Letterera — Siwa a disease ), nek-ry hemolitic anemias. The Nek-ry diseases proceeding with increase in a spleen can begin at children's age, napr, the tromboflebitichesky splenomegaly which is followed by the phenomena of a hypersplenism.

As well as at adults, at children the spleen increases at many acute and hron. infectious diseases. At the same time sometimes S. is the main symptom. So, at inf. S.'s mononucleosis as the isolated symptom can remain persistently for many years after a disease; at inborn syphilis (see) in the first months of life the dense spleen is an important diagnostic character; at acute forms of miliary tuberculosis at children, especially chest age, significant increase in a spleen is observed.

At the nek-ry diseases which are followed by S. an operative measure, napr is shown, at Minkowski's disease — Shoffara (see. Hemolitic anemia ), Verlgof's diseases (see. Werlhof's disease ).

Bibliography: Bart I. A spleen, the lane with it., Budapest, 1976; To and p r I., etc. Limforetikulyarny diseases, the lane, with English, page 204, M., 1980; In ii of k i H. and. and. Milz und Antikorperbildung, Schweiz, med. Wschr., S. 1351, 1974; Dame she to W. Splenomegaly, a problem in differential diagnosis, Med. Clin. N. Amer. v. 41, p. 1357, 1957; Ford W. L. a.Nieuwen-h u i s P. The spleen and lymphocytes, Schweiz, med. Wschr., S. 1348, 1974.

P. Mokeeva