α-重链病是分泌异常IgA的B淋巴细胞系统增殖性疾病,临床有肠型与呼吸道型两种,首例由Sle-gmann报告,并因其常合并小肠淋巴瘤,且多见于地中海地区各国而被命名“地中海腹部淋巴瘤,”据目前研究,现已在世界各地相继发现相同病例。W.H.O提议代之以Immuno-Proliferative Small IntestinalDisease,简作IPSLD,译名免疫增殖性小肠病,肠型α-HCD不一定进入淋巴瘤阶段,其合并存在的小肠淋巴瘤常常是B细胞转化的免疫母细胞肉瘤型淋巴瘤,在发展成淋巴瘤时,细胞丧失分泌α-重链的功能,此 Alpha-heavy chain disease is an abnormal IgA B lymphocyte system proliferative disease, clinical intestinal and respiratory two, the first reported by Sle-gmann, and often associated with intestinal lymphoma, and more common in the Mediterranean region Countries have been named “Mediterranean abdominal lymphoma,” according to current research, the same cases have been found around the world. WHO proposed to replace them with Immuno-Proliferative Small Intestinal Disease, abbreviated as IPSLD, the name of immune proliferative small bowel disease, enteric α-HCD does not necessarily enter the lymphoma stage, the combined presence of small intestine lymphoma is often B cell transformed immune cells Sarcomatoid lymphoma, in the development of lymphoma, the loss of secretion of α-heavy chain cells, this