Washington K, Stenzel T T, Buckley R H, Gottfried M R
Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA.
Am J Surg Pathol. 1996 Oct;20(10):1240-52. doi: 10.1097/00000478-199610000-00010.
Review of the medical records of 43 patients with common variable immunodeficiency (CVID) and 23 patients with X-linked agammaglobulinemia (XLAG) revealed a high incidence of chronic gastrointestinal complaints, most commonly diarrhea. Thirty-eight biopsies, four small-bowel resection specimens, and one autopsy from 10 patients with CVID and one patient with XLAG showed a wide range of abnormalities. A pattern resembling acute graft-versus-host disease, with apoptotic bodies and lymphocytes in crypts, was seen in the stomach (four patients), small bowel (three patients), and colon (three patients). Small-bowel specimens from three CVID patients with malabsorption showed mild to severe villous atrophy. Three CVID patients had Giardia in biopsies. Two cases of small bowel lymphoma associated with nodular lymphoid hyperplasia were identified in CVID patients. One patient's small bowel contained foamy histiocytes in the lamina propria, resembling Whipple's disease or chronic granulomatous disease, with numerous apoptotic bodies in crypts. Ultrastructurally, the histiocytes contained cellular debris. The patient with XLAG had recurrent fissuring necrosis of small bowel resembling Crohn's disease; a patient with CVID had colitis with features similar to ulcerative colitis. Poorly formed granulomas were seen in the stomach (one CVID patient) and the colon (two CVID patients). Lymphocyte populations were dominated by T cells; B cells were scarce except in lymphoid follicles in CVID patients with nodular lymphoid hyperplasia. Patients with CVID and XLAG manifest a spectrum of abnormalities in the gastrointestinal tract, with patterns superficially resembling graft-versus-host disease, inflammatory bowel disease, and Whipple's disease, but often lacking some of the diagnostic features of the diseases. Many of the CVID patients with chronic gastrointestinal complaints (62%) also had evidence of autoimmune phenomena, suggesting that in some patients the inflammatory process in the gastrointestinal tract has an autoimmune component.
对43例普通可变免疫缺陷（CVID）患者和23例X连锁无丙种球蛋白血症（XLAG）患者的病历审查显示，慢性胃肠道不适的发生率很高，最常见的是腹泻。对10例CVID患者和1例XLAG患者进行的38次活检、4份小肠切除标本及1次尸检显示出广泛的异常情况。在胃（4例患者）、小肠（3例患者）和结肠（3例患者）中可见一种类似于急性移植物抗宿主病的模式，隐窝中有凋亡小体和淋巴细胞。3例有吸收不良的CVID患者的小肠标本显示有轻至重度绒毛萎缩。3例CVID患者的活检标本中有贾第虫。在CVID患者中发现2例与结节性淋巴样增生相关的小肠淋巴瘤。1例患者的小肠固有层中有泡沫状组织细胞，类似于惠普尔病或慢性肉芽肿病，隐窝中有大量凋亡小体。超微结构下，组织细胞含有细胞碎片。XLAG患者有类似于克罗恩病的小肠复发性裂隙性坏死；1例CVID患者有类似于溃疡性结肠炎特征的结肠炎。在胃（1例CVID患者）和结肠（2例CVID患者）中可见形成不良的肉芽肿。淋巴细胞群体以T细胞为主；除了有结节性淋巴样增生的CVID患者的淋巴滤泡外，B细胞稀少。CVID和XLAG患者在胃肠道表现出一系列异常，其模式表面上类似于移植物抗宿主病、炎症性肠病和惠普尔病，但往往缺乏这些疾病的一些诊断特征。许多有慢性胃肠道不适的CVID患者（62%）也有自身免疫现象的证据，这表明在一些患者中，胃肠道的炎症过程有自身免疫成分。
