Illness heavy and - chains and a lymphoma of a small bowel
The illness and - chains — a limfoproliferativny disease (one of lymphoma types), is characterized a diffusion infiltratsiy mucous membrane of a small bowel and lymph nodes of a mesentery by the plasmocytes cosecreting the pathological immunoglobulin deprived of light chains and containing defective heavy chains.
Epidemiology The illness and - chains is for the first time described by E. S. Franclin and soavt. in 1964. Now in literature more than 200 cases of this disease which received also the name of "the Mediterranean abdominal lymphoma" as often it is found in this region are described. Also other options of an illness of heavy chains are described (m chains, delta chains and a lambda chains).
Etiology and pathogeny It is supposed that at the heart of an illness and - chains the primary immunodeficience connected with inborn disturbance of synthesis of immunoglobulins lies. Also the hypothesis of possibility of an illness and - chains owing to a long invasion the intestinal microorganisms calling the raised products of IgA expresses. It is impossible to exclude also possibilities of the specific virus agent as etiological factor.
Consecutive histologic researches showed that at an illness and - chains there is a transition from the high-differentiated limfoproliferativny process to the blast cellular proliferation inherent to lymphoma. Therefore now believe that the illness and - chains and a lymphoma is represented by various stages of one process.
Classification Classification is absent.
Approximate formulation of the diagnosis: 1. An illness and - chains. A syndrome of a hyper catabolic exudative enteropathy with the expressed hypoproteinemia, a cachexia. 2. An illness and - chains. The chronic quickly increasing enteric impassability.
Preliminary diagnosis The leading clinical symptoms of an illness and - chains are the plentiful watery chair, considerable loss of body weight, an abdominal pain, ascites, hypostases, spasms. The symptom of "drum sticks" is often observed. In process of progressing of an illness perforation, invagination with development of impassability and peritonitis are possible.
Characteristic laboratory symptoms of an illness — a steatorrhea, a considerable hypoproteinemia, decrease in level of cholesterol, potassium and calcium, iron in blood serum, anemia. Typical radiological signs of a lymphoma are a rough relief because of a thickening of a mucous membrane, existence of strictures and defects of filling owing to a hyperplasia of lymphoid follicles in a submucosal layer, and also polyrecurrence of contours because of the increased lymph nodes.
Verification of the diagnosis, differential diagnosis The leading role in diagnosis of an illness and - chains belongs to a biopsy of a mucous membrane of a small bowel: the massive cellular infiltration plasmocytes and lymphocytes with impurity of macrophages comes to light. Also the flattening vorsin a small bowel is noted, but unlike a Gee's disease the superficial epithelium is well kept. Emergence of atypical cells testifies to a process zlokachestvennost.
Not less important role is played also by immunological research of blood serum and other secrets (saliva, intestinal juice) in which find pathological protein — heavy and - chains. Apply an immunoelectrophoresis method with monospecific antiserum to IgA. At the same time there is an abnormal precipitation line in a zone of a1-a2-globulins.
Clinical symptoms of an illness and - chains are in many respects similar to other diseases which are followed by a heavy syndrome of the broken absorption and a hyper catabolic exudative enteropathy (primary immunodeficiences, an intestinal lymphangiectasia, Whipple's illness, etc.).
In each case, proceeding with heavy malabsorption, it is necessary to conduct surely histologic research of a mucous membrane of a small bowel, and also immunological research of blood serum, With their help diagnose a lymphoma or its subtype — the illness and - chains, is excluded by primary variable immunodeficience, Whipple's illness and a lymphangiectasia.
Treatment, forecast At an illness and - chains apply antibiotics, corticosteroid drugs, cytostatic means and radiation therapy. The tetracycline and ampicillin accepted inside in usual doses are effective. However remission usually does not happen long. In this regard along with antibiotics complex therapy by cytostatic drugs (Cyclophosphanum, corticosteroids) by means of which it is possible to reach longer remission is necessary.
If there are signs of transformation of an illness and - chains in a lymphosarcoma, radiation therapy and polychemotherapy are necessary.
The forecast at an illness and - chains generally adverse as, despite a possibility of long remissions (till 2 flyings), the disease recurs. When the illness is transformed to a lymphoma, the forecast becomes bad. The death occurs from a cachexia, infectious complications and a rvsprostraneniye of tumoral process.