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Alpha‐chain disease. Report of a case from taiwan
Author(s) -
Shih LeeYung,
Liaw ShiumnJen,
Hsueh Swei,
Kuo TsengTong
Publication year - 1987
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19870201)59:3<545::aid-cncr2820590331>3.0.co;2-c
Subject(s) - medicine , pathology , lymphoplasmacytic lymphoma , lamina propria , immunoperoxidase , villous atrophy , mesenteric lymph nodes , malabsorption , lymphoma , biopsy , infiltration (hvac) , gastrointestinal tract , disease , gastroenterology , antibody , waldenstrom macroglobulinemia , immunology , coeliac disease , spleen , epithelium , monoclonal antibody , physics , thermodynamics
The occurrence of alpha‐chain disease (α‐CD) in a Chinese patient, a resident of Taiwan, is reported. The patient's clinical features were characterized by severe malabsorption and ran a rapidly deteriorated course. Duodenal biopsy showed total villous atrophy with a heavy infiltration of the lamina propria by lymphoplasmacytic cells. Colonic biopsy revealed immunoblastic lymphoma. α‐CD protein was demonstrated in the patient's serum and concentrated urine by the immunoselection method, and also detected in the cellular infiltrate by immunoperoxidase technique. Postmortem examination disclosed that the lymphoma involved not only the entire gastrointestinal tract and mesenteric nodes, but also the retroperitoneal and peripheral lymph nodes. Immunoperoxidase studies support the concept of a common clonal origin of the lymphoplasmacytic proliferation and immunoblastic lymphoma in α‐CD.