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小肠弥漫性滤泡性淋巴组织增生,无原发性免疫球蛋白缺乏症。

Diffuse follicular lymphoid hyperplasia of the small intestine without primary immunoglobulin deficiency.

作者信息

Rambaud J C, De Saint-Louvent P, Marti R, Galian A, Mason D Y, Wassef M, Licht H, Valleur P, Bernier J J

出版信息

Am J Med. 1982 Jul;73(1):125-32. doi: 10.1016/0002-9343(82)90938-x.

Abstract

Three cases of follicular lymphoid hyperplasia extending to the whole length of small intestine are reported in three young adult patients of low economic status. The disease was revealed by chronic diarrhea with malabsorption and/or protein-losing enteropathy. In one patient, all transitional patterns were found between the hyperplastic follicles and a small intestinal multicentric centrocytic-centroblastic lymphoma. No abnormalities in humoral or cellular immunity were found in the three patients. In particular, serum immunoglobulins (except IgG in one case) and plasma cell populations of small intestinal lamina propria were normal. Diffuse follicular lymphoid hyperplasia of the small intestine in the absence of primary immunoglobulin deficiency appears to be a rare condition associated with (or leading to) intestinal malignant lymphoma in most cases. Patients usually belong to the same populations as those suffering from alpha-chain disease.

摘要

报告了3例经济状况较差的年轻成年患者,其滤泡性淋巴组织增生累及小肠全长。该病表现为慢性腹泻伴吸收不良和/或蛋白丢失性肠病。在1例患者中,发现增生性滤泡与小肠多中心中心细胞-中心母细胞淋巴瘤之间存在所有过渡模式。3例患者的体液免疫或细胞免疫均无异常。特别是,血清免疫球蛋白(1例患者的IgG除外)和小肠固有层的浆细胞群体均正常。在无原发性免疫球蛋白缺乏的情况下,小肠弥漫性滤泡性淋巴组织增生似乎是一种罕见疾病,在大多数情况下与肠道恶性淋巴瘤相关(或导致肠道恶性淋巴瘤)。患者通常与患α链病的人群相同。

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