Martin I G, Aldoori M I
Department of Surgery, Pontefract General Infirmary, Pontefract, UK.
Br J Surg. 1994 Jan;81(1):20-4. doi: 10.1002/bjs.1800810107.
Gastrointestinal lymphoma, uncommon in the West, is far more prevalent in developing countries where it falls into two groups: 'Western'-type lymphomas, similar to those seen in developed countries, and the so-called Mediterranean-type lymphoma. It is now accepted that Mediterranean lymphoma represents, in the majority if not in all cases, the late stage of alpha heavy chain disease (alpha-HCD). This disease is characterized by abnormal secretion of an immunoglobulin fragment; alpha-HCD and Mediterranean lymphoma constitute two ends of a spectrum of pathology now classified as immunoproliferative small intestinal disease (IPSID). IPSID is associated predominantly with poor socioeconomic conditions; patients present with progressive malabsorption in the second and third decades of life. Diagnosis is established by small bowel biopsy, with or without high serum levels of the alpha heavy chain protein. Treatment consists of an initial staging laparotomy, with debulking of lymphomatous deposits if appropriate, followed by chemotherapy or radiotherapy. Overall prognosis is poor but the recent use of doxorubicin-based chemotherapy offers some hope for the future.
胃肠道淋巴瘤在西方并不常见，但在发展中国家更为普遍，可分为两类：“西方”型淋巴瘤，与发达国家所见的相似，以及所谓的地中海型淋巴瘤。现在人们认为，地中海淋巴瘤在大多数情况下（如果不是所有情况）代表α重链病（α-HCD）的晚期。这种疾病的特征是免疫球蛋白片段的异常分泌；α-HCD和地中海淋巴瘤构成了现在归类为免疫增殖性小肠疾病（IPSID）的病理谱的两端。IPSID主要与社会经济条件差有关；患者在生命的第二个和第三个十年出现进行性吸收不良。通过小肠活检确诊，血清α重链蛋白水平可高可低。治疗包括最初的分期剖腹手术，如有必要，清除淋巴瘤沉积物，然后进行化疗或放疗。总体预后较差，但最近使用基于阿霉素的化疗为未来带来了一些希望。
