免疫增殖性小肠病多见于地中海沿岸地区,其他地区罕见。临床上以腹泻、小肠吸收不良综合征为主要表现。该病以分泌一种仅含α-重链结构而缺少轻链部分的异常免疫球蛋日A(IgA)为特点故又称为α-重链病。部分病人可发展成为一种侵及大部小肠的原发性小肠恶性淋巴瘤——地中海淋巴瘤。 Proliferation of immune small bowel disease more common in the Mediterranean coast, rare in other areas. Clinically, diarrhea, intestinal malnutrition syndrome as the main performance. The disease, also known as alpha-heavy chain disease, is characterized by the secretion of an abnormal immunoglobulin A (IgA) that contains only the alpha-heavy chain structure but lacks the light chain portion. Some patients can develop into a small intestinal invasion of most of the primary small intestinal lymphoma - lymphomas.