Heavy alpha-chain disease
Lorie, N.Iu.; Ekisenina, N.I.
Klinicheskaia Meditsina 59(4): 32-35
1981
ISSN/ISBN: 0023-2149 PMID: 6787299 Document Number: 175805
A case of H .alpha.-chain disease is described. This is the rarest disease affecting the IgA system and characterized by proliferation of B-cells of the membrana propria of the small intestine and mesenteric lymph nodes. These cells synthesize pathological protein devoid of L chains and containing defective chains devoid of a part of the Fd-fragment. Clinically HCD-.alpha. is characterized by severe diarrhea with the pronounced syndrome of malabsorption, progressive cachexia and the development of lymphosarcoma in the absence of treatment. Data are presented on the possible remission as a result of a long-term oral antibiotic therapy or a combination of antibiotics and cyclophosphane. Laparotomy is necessary and valuable for the diagnosis of diarrhea of an unclear genesis. In the case described the clinical picture of HCD-.alpha. was typical, and the diagnosis was confirmed by characteristic X-ray, histological and immunochemical indices.