Mrabti Hind, Raiss Ghizlane, Raissouni Soundouss, Tazi El Mehdi, Inghaouen Hanane, El Ghissassi Ibrahim, Errihani Hassan
Institut national d'oncologie, service d'oncologie médicale, Rabat 10100, Maroc.
Presse Med. 2011 Nov;40(11):995-1000. doi: 10.1016/j.lpm.2011.02.021. Epub 2011 Mar 31.
Immunoproliferative small intestinal disease (IPSID), also known as alpha chain disease, is a rare disease. In the recent WHO classification of hematopoietic and lymphoid tissue, IPSID is considered as a variant of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma. Campylobacter jejuni is a specific pathogen, found to be related to IPSID. Diagnosis is based on histology and immunochemistry (± fluorescent in situ hybridization), with presence of many variable levels of abnormal immunoglobulin in the serum, identified to be truncated alpha-heavy chains. Early-stage disease is treated by antibiotics (tetracyclines). Chemotherapy is recommended up front for patients with advanced disease at presentation or refractory to antibiotics. The chemotherapy schedule used is the CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen.
免疫增殖性小肠疾病（IPSID），也称为α链病，是一种罕见疾病。在世界卫生组织最近的造血和淋巴组织分类中，IPSID被视为结外黏膜相关淋巴组织（MALT）淋巴瘤的一种变体。空肠弯曲菌是一种特定病原体，被发现与IPSID有关。诊断基于组织学和免疫化学（±荧光原位杂交），血清中存在许多不同水平的异常免疫球蛋白，经鉴定为截短的α重链。早期疾病用抗生素（四环素）治疗。对于就诊时处于晚期或对抗生素耐药的患者，建议 upfront 进行化疗。所使用的化疗方案是CHOP（环磷酰胺、长春新碱、阿霉素和泼尼松）方案。
