Alpha-chain disease. Report of a case from Taiwan

The occurrence of alpha-chain disease (alpha-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. alpha-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 alpha-CD.