[Alpha heavy chain disease: a clinical study of 3 cases and review of the literature]

Abstract

Alpha-chain disease is a rare and interesting form of small intestinal immunoproliferative disease. In this paper we present three personal observations with different clinical manifestations. The first patients was admitted to the hospital because of weight loss, feet and ankles edema, and was complicated by intestinal occlusion that required an extensive enteric resection in urgency. After the operation, chemotherapy was carried out with good results at a five-years follow-up period. The second case, characterized by diffuse abdominal pain, diarrhea, weight loss and peripheral edema, was complicated by ileal perforation. An intestinal resection associated to mesenteric lymphnodes removal was performed. Chemotherapy and antibiotics were done successfully at a four-years follow-up period. In the last patient, admitted to our hospital because of abdominal discomfort, diarrhea and weight loss, a laparotomy was performed for confirming the diagnostic suspect and staging the disease. Only antibiotic therapy was done with good results at one-year follow-up period. We review and discuss the major clinical features and the main aspects of etiology, epidemiology, pathogenesis, pathology and therapy of this disease.