From Big Medical Encyclopedia


the V-limfoproliferativnykh group of diseases, character from at a yushcha hsya

disturbance of synthesis of immunoglobulins and emergence in blood and urine of the atypical protein presented by fragments heavy it is valuable immunoglobulins.

According to the clinical and morphological characteristic of the disease of heavy chains (DHC) are close to a lymphosarcoma (see) or to a chronic lymphoid leukosis (see Leukoses). Their distinctive feature is existence in blood serum and (or) in urine of a paraprotein — the abnormal protein presented by dimeasures of the delegated heavy chains of immunoglobulins of one of classes — at, and or ji (see Immunoglobulins). According to it distinguish BTTs-u, BTTs-ss and BTTs-ju.

BTTs-u (Franklin disease) it is for the first time described in 1964 by Franklin (E. S. Franklin) et al. Now in the world literature there are messages only on several tens such patients. The disease occurs preferential at men. The most characteristic symptom — the local or generalized, sometimes alternating lymphadenopathy; at the same time Valdeyer's ring (see Almonds) is often involved in process in this connection hypostasis and a hyperemia of a soft palate and a uvula develops. Fever, and also sple-no-and a hepatomegalia is sometimes noted (see. Hepatolienal syndrome). For BTTs-u manifestations are rare Shegren's syndrome (see Shegren a syndrome), a pseudorheumatism (see), pulmonary fibrosis (see the Pneumosclerosis), a myasthenia (see), a thyroiditis (see) and a tumor of a thyroid gland (see).

At a lab. a blood analysis, as a rule, reveal anemia, a leukopenia with a relative lymphocytosis, existence of atypical lymphocytes or plasmocytes, often an eosinophilia (see), thrombocytopenia) (see) and the accelerated ROE, quite often a hyperuricemia (see. Uric acid).

Concentration of crude protein in blood serum is normal or is lowered (see the Proteinemia); at an electrophoresis find, as a rule, a hypoalbuminemia and the M-gradient in a zone of R-globulins or Y-globulins (see fig. 7 to St. Multiple myeloma, t. 15, Art. 151). The M-gradient at small concentration of a paraprotein can not come to light. In such cases the major diagnostic importance gets a research of urine. For BTTs-u the constant and usually considerable proteinuria is characteristic (see); in this case at an electrophoresis all protein of urine (uroparaprotein) migrates in a zone at or P according to a serumal paraprotein. At an immunoelectrophoresis (see) a paraprotein, both serumal, and being in urine, does not react with antiserums against light chains x and And, it gives precipitation

with an antiserum against heavy chains at, but not against GA-frag-mentov that indicates, on the one hand, absence in a molecule of a paraprotein of light chains and, on the other hand — on deficiency heavy. The structural analysis of a paraprotein at BTTs-u showed that it represents dimeasures of two heavy chains with partial deletion of Fd-fragments and (or) hinged area that, apparently, is a consequence of genovariation and the synthesis of a molecule of immunoglobulin broken in this regard; pier. the weight (weight) of monomer of 25 Ltd companies — 58000 (coefficient of sedimentation of 2,8 — 4,0 S).

In limf, nodes diffusion or focal infiltration of fabric lymphoid and plasmocytes with impurity of eosinophils is noted. During the involvement in process of marrow in it the similar picture is observed.

BTTs-a it is described in 1969 Mr. Selig-semolina (M. by Seligmann) with sotr. The disease is widespread among residents of North Africa and the Middle East; in a crust, time meets also in other geographical zones; persons of both sexes are ill, it is preferential at youthful age. J. W. Stoop with sotr. in 1971 described two children with this BTTs form, at to-rykh lungs were affected. BTTs-a it is observed, apparently, more often than BTTs-u. To a crust, time knows apprx. 150 observations. A wedge, manifestations (wearisome diarrhea, a steatorrhea, the progressing exhaustion), same, as at a syndrome of the broken intestinal absorption # (see Maljabsorbtion a syndrome). BTTs-a unlike BTTs-u proceeds, as a rule, without generalization. Disturbance of absorption in a small bowel leads to Hypaque of l of a tsiyemiya, a hypophosphatemia, a hypo lipidemiya,

a hypoglycemia, a hypoalbuminemia, to increase in maintenance of an alkaline phosphatase, and also to a gipokaltsi-uriya. At a biopsy of a mucous membrane of proximal department of a small bowel diffusion infiltration of own plate lymphatic, plasmatic and reticular macrophages comes to light. Similar gistol. the picture is noted in increased mezenterialny limf, nodes.

Gemogramma (see), except for moderate anemia, usually within norm. Concentration of crude protein in blood serum is lowered or is normal. Classical M-gra-diyent (in the form of a narrow strip) on an elekt-roforegramma usually is absent, however the wide strip (spot) stretching from a2-to ^-globulins and including these fractions is often observed. By means of an immunoelectrophoresis with monospecific antiserums find an arch of precipitation of a paraprotein with an antiserum against heavy a-chains, but do not reveal an arch of precipitation against heavy chains of immunoglobulins of other classes and against light chains. More detailed characteristic of this paraprotein is complicated because of his tendency to polymerization. The structural analysis of a paraprotein at BTTs-a showed that it represents dimeasures heavy and - the chains which are characterized by big variability a pier. the weight (from 34 000 to 56 000), tendency to polymerization, presence of the full Fc-frag-cop and heterogeneity in the sequence of the amino-acid remains in an iolipeptidny chain; the question

of safety of a Fd-fragment remains open. The paraprotein revealed in urine, saliva and in contents of a small bowel has the same properties, as serumal.

BTTs-(y it is for the first time described in 1970. F. A. Forte with sotr.; further there were messages

on several patients (apprx. only 20 observations). Wedge, a picture and a current of this BTTs form are close to a chronic lymphoid leukosis (see Leukoses), however increase limf, nodes, a spleen and a liver at BTTs-jm is insignificant, and at nek-ry patients is absent. At a number of persons the system amyloidosis (see), patol comes to light. fractures of bones (see Changes, pathological). In marrow, and also in limf, nodes the hyperplasia of small (mature) lymphocytes and vacuolated plasmocytes is defined. The picture of blood is, as a rule, typical for a chronic subleukemic lymphoid leukosis; certain patients can have a quantity of leukocytes normal or is lowered, and the leukocytic formula is not changed. Concentration of crude protein in blood serum usually normal or is lowered. At an electrophoresis reveal a hypoalbuminemia, hypogamma globulin-miyu, and in nek-ry cases — emergence of abnormal strips of a paraprotein in various positions. On elektroforegramma find quickly migrating component reacting with an antiserum against heavy jn-chains. In addition to heavy jLi-chains, in blood serum sometimes reveal also free light chains of immunoglobulins. In urine the high or moderately increased concentration of protein, as a rule, is defined, to-ry it is presented by free light chains of immunoglobulin of one type in this connection the albumin test of Bens-Jones in urine, as a rule, happens positive (see Bens-Jones of squirrels). About structure of abnormal protein at BTTs-jn a detailed information is absent. It is known that it is presented by a dimer of heavy ju-chains about a pier. weighing 50 000 — 55 000 also has tendency to polymerization. Believe that in polypeptide chains of this abnormal protein there is partial deletion of Fc-, and it is possible also Fd-fragments. Unlike БТЦ-^ and BTTs-a at BTTs-ji probably takes place not only defect of synthesis, but also defect of assembly of the molecule IgM.

Signs to an imm but scarce states are characteristic of all BTTs forms (see. Immunological nedostatochnosti), to-rye are noted at repeated inf. complications; along with a tumoral progression (see the Progression of a tumor) inf. complications are the main reasons for death. At BTTs-a death often comes in connection with exhaustion and disturbance of a water salt metabolism.

The diagnosis of BTTs on the basis a wedge, pictures and data a lab. researches it is complicated; it is finalized by means of the immunochemical methods revealing in blood serum and (or) in urine the paraproteins presented isolated heavy at - and - or { Y-chains of immunoglobulins.

Treatment of all BTTs forms consists in purpose of cytostatic means, corticosteroid hormones, transfusions of eritrotsitny weight (for more details see the Lymphosarcoma) \at BTTs-a, in addition to chemotherapy and corticosteroid hormones, the leading role is played by correction of a syndrome of the broken intestinal absorption (see Maljabsorbtion a syndrome) and parenteral food (see). At all BTTs forms a certain value has use of immunocorrective means, napr, native plasma, levamisole (decarice) for prevention and antibiotics for treatment inf. complications.

Forecast adverse.

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page 32, 1981; Frangdronge B. Wa

the riant of a heavy chain, proteins synthesized at «a disease of heavy chains» in book: Immunoglobulins, under the editorship of G. Litmen and R. Goode, lane with English, page 343, M., 1981; I in about r to about in with to and y L. I. Paraproteinemia and its clinical value, page 120, Riga, 1981; Chernokhvosto-V an E. V. a. German G. P. Immunochemical study in two cases of alpha chain disease, Folia haemat. (Lpz.), v. 107, p. 757, 1980; Forte F. A. a. o. Heavy chain disease of the (gamma M) type, Blood, v. 34, p. 137, 1970; Frangi one B. Franklin E. G. Heavy chain diseases, clinical features and molecular significance of the disordered immunoglobulin structure, Sem.; Haematol., v. 10, p. 53, 1973, bibliogr.; F r a n k 1 i n E. C. a. o. Heavy chain disease, A new disorder of serum gamma-globulins, Amer. J. Med., v. 37, p. 332, 1964; Stoop J.

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