Arista-Nasr J, Hernández-Pando R
Departamento de Patología, Instituto Nacional de la Nutrición Salvador Zubirán, México, D.F.
Rev Invest Clin. 1993 May-Jun;45(3):275-80.
The clinico-pathological characteristics of a 22 years old mestizo mexican patient with immunoproliferative small intestinal disease (IPSID) are described. The patient coursed with chronic diarrhea, malabsorption, and a 30 kg weight loss. The laboratory tests revealed hypokalemia, hypocalcemia, hypoalbuminemia and decreased levels of immunoglobulins. The histological changes in the endoscopic biopsies included a dense lymphoplasmacytic infiltrate in the lamina propria that produced vellosity blunting and distortion of the crypts. The immunohistochemical analysis disclosed that almost every cell was positive to heavy chains and negative to light chains. The radiological studies showed thickening of the intestinal folds and mesenteric adenomegalies. In the full thickness biopsy of the yeyunum, in addition to the lymphoplasmacytic infiltrate, a high grade malignant lymphoma infiltrating the regional lymph nodes was documented. An ultrastructural study revealed viruses that resembled the morphology described for the Epstein-Barr virus. The main clinico-pathological findings of other eight mexican patients with IPSID previously reported are commented. It is concluded that the IPSID is a rare disease in our country but it can be found sporadically.
描述了一名22岁患有免疫增殖性小肠疾病(IPSID)的混血墨西哥患者的临床病理特征。该患者病程中有慢性腹泻、吸收不良以及体重减轻30公斤。实验室检查显示低钾血症、低钙血症、低白蛋白血症以及免疫球蛋白水平降低。内镜活检的组织学变化包括固有层密集的淋巴细胞和浆细胞浸润,导致绒毛变钝和隐窝扭曲。免疫组化分析显示几乎每个细胞重链阳性而轻链阴性。放射学研究显示肠襞增厚和肠系膜淋巴结肿大。在空肠全层活检中,除淋巴细胞和浆细胞浸润外,还记录到高级别恶性淋巴瘤浸润区域淋巴结。超微结构研究发现了形态类似于爱泼斯坦-巴尔病毒的病毒。对先前报道的其他8例墨西哥IPSID患者的主要临床病理发现进行了评论。得出结论,IPSID在我国是一种罕见疾病,但可散在发现。