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Diseases of heavy chains

Diseases of heavy chains represent the limfoproliferativny diseases with various morphology and clinical manifestations which are characterized by proliferation of the lymphoid elements of the B-phenotype synthesizing and cosecreting heavy chains.

Epidemiology
Diseases of heavy chains are rare diseases, only about 50 well documentary cases among which there are diseases of heavy chains of all subclasses of IgG are published; the disease occurs mainly at men more young than 40 years and at children of various racial accessory.

The illness and - heavy chains, meeting more often than an illness at - heavy chains, was for the first time found among inhabitants of the Mediterranean Basin and described under the name "Mediterranean lymphoma". The intestinal form of an illness and - heavy chains meets in North Africa, in the Middle East, in the south of Italy, in the basin of the Mediterranean Sea. It is registered, as a rule, in regions with high incidence of intestinal infections which, creating conditions to the raised antigen challenge, can play primary role in formation of an illness.

Etiology and pathogeny
For an explanation of a pathogeny of an illness of heavy chains the hypothesis of defeat of the gene controlling formation of one or several segments of heavy chains when cells formed also light chains is offered, but could not connect them to heavy; according to other hypothesis attach significance to defeat of the factors controlling 2 genes (for cases without formation of light chains).

Abnormal proteins at diseases of heavy chains are formed in pathological plasmocytes. Emergence only of heavy chains is explained with genovariation with possible deletion of the gene defining synthesis of heavy chains. Very seldom heavy chains of a mu find in urine. Only use of specific antiserums allows to reveal heavy chains and to prove their isolation from light chains. Heavy chains differ in homogeneity, always belong only to one of the known subclasses of immunoglobulins. Heavy chains and and a mu possess high ability to polymerization, their molecular weight is less, than normal analogs.

Classification
Allocate separate diseases of heavy chains of a form depending on type of heavy chains. Clinically diseases of heavy chains meet quickly progressing current and slow development of characteristic symptomatology.

Approximate formulation of the diagnosis:
1. An illness and - heavy chains, slowly progressing with insignificant increase in a liver and spleen, peripheral lymph nodes, almonds, anemia, leucio-and thrombocytopenia, the increased quantity limfoidiy both plasmocytes in blood and marrow.
2. An illness at - heavy chains, quickly progressing, with diarrhea, the migrating arthralgias, a hyperplasia of a liver, mezenterialny lymph nodes with proliferation in them and in marrow of plasmocytes a pancytopenia, an immunodeficient syndrome, left-side nizhnedolevy bronchial pneumonia, a cachexia.

Clinic
The illness at - heavy chains is quite often designated in literature as "an illness of Franklin". Her clinical picture is characterized by fast progressing, a hyperplasia of peripheral, mediastinal, retroperitoneal lymph nodes. A ring in the form of a tumor of almonds, a uvula, the sky, and also the liver, a spleen, a thyroid gland are surprised valdeyerovo. Fever is often caused by the infectious complications which are quite often a cause of death. Histologically find proliferation of lymphoid cells of various degree of a maturity, plazmokletochny infiltrates.

Researches of a gemogramma reveal anemia, at 1/3 patients — the leukopenia which is usually caused by a granulocytopenia, atypical lymphoid and plasmocytes, sometimes an eosinophilia at a half of patients — thrombocytopenia. SOE is often raised. The hyperuricemia is observed. In a miyelogramma the maintenance of plasmocytes and (or) lymphocytes and reticular macrophages is usually increased.

The diagnosis of an illness at - heavy chains is based on detection in serum and (or) urine of an abnormal protein. Content of crude protein in blood serum is usually normal or reduced.
The illness at - heavy chains quite often arises at the persons who earlier had autoimmune diseases: system lupus erythematosus, pseudorheumatism, Sjegren's syndrome, pulmonary fibrosis, myasthenia, thyroiditis, hemolitic anemia. At other patients identification of the M-component is preceded by a polyclonal gammapathy.

The illness and - heavy chains is characterized by chronic diarrhea, weight loss, pallor of integuments, hypostases, disturbance of a phosphorus-calcium exchange, baldness, the migrating arthralgias, incidental fever, pristupoobrazny abdominal pains, vomiting. In the developed stage of a disease note a hyperplasia of mezenterialny lymph nodes, sometimes — a hepatomegalia. Disturbance of absorption is expressed in a hypocalcemia, a hypocalciuria, a hypophosphatemia, increase of level of alkaline phosphatases, decrease in level of lipids, a prothrombin, blood sugar.

Radiological reveal changes of a mucous membrane of a stomach and a large intestine (disorganization of folds of a mucous membrane with alternation of zones of narrowing and expansion). At histologic research on all mucous membrane of a small bowel find diffusion proliferation of lymphocytes and plasmocytes with atipizm signs, an atrophy of fibers, reduction of crypts. Cells of an epithelium are not damaged. In giperplazirovanny mezenterialny lymph nodes the picture of proliferation of plasmocytes lympho-or reticulosarcomas comes to light. There are single descriptions of an illness with a marrow infiltration. The disease quickly progresses, the cachexia, abdominal and other complications are a proximate cause of death.

At an illness of a mu heavy chains for a long time patients have no increase in peripheral lymph nodes, the liver and a spleen early enough increase, there is an amyloidosis, pathological changes. In marrow find a lymphocytosis, plasmocytes with pathological vacuolation.

Verification of the diagnosis
Diagnosis is based on identification of the monoclonal immunoglobulin containing a fragment only of a heavy chain of one of the main classes of immunoglobulins. It is possible only at an immunochemical blood analysis and (or) urine with use of antiserums to heavy chains.

Treatment
Doctors have no now rather effective remedies of treatment of diseases of heavy chains yet. Local radiation of a spleen and lymph nodes gives fast, but short-term improvement. Alkylating agents are also poorly effective. At certain patients the favorable answer to Prednisolonum or Vincristinum is noted. Attempts of the combined chemotherapy by the principles of treatment of a chronic lymphoid leukosis, malignant nekhodzhkinsky lymphoma are made. In the presence of an immunodeficient syndrome purpose of immunocorrective means is justified. Treatment cases at an illness are described and - heavy chains as a result of prolonged use of antibiotics. In recent years at diseases of heavy chains use a plasma exchange.

 
"Angiokeratoma   Bowen's illness"