Zarrabi M H, Rosner F
Am J Med Sci. 1976 Jul-Aug;272(1):101-19. doi: 10.1097/00000441-197607000-00013.
A 20-year-old Persian man with Middle Eastern lymphoma is described, and 84 additional cases from the literature are reviewed. Basically, the disease is a malignant lymphoma which involves the upper small intestine (duodenum and proximal jejunum). It is associated with clubbing of the fingers, abdominal pain, weight loss, diarrhea, vomiting, and malabsorption, and frequently occurs in a younger age group than "Western Hemisphere" intestinal lymphoma. Some patients also have alpha heavy chain disease. The sex ratio is equal, and the disease occurs only in Middle Eastern and North African Moslems and Jews. Upper gastrointestinal radiographs are frequently diagnostic, and per oral small intestinal biopsy is nearly always diagnostic. Pathologically, the following features are characteristic for Middle Eastern lymphoma: partial or total villous atrophy with only mildly abnormal surface epithelium, sparsity of crypts, lymphatic dilatation, and infiltration of lamina propria by pleomorphic mononuclear cells which pepetrate the muscularis mucosa. The etiology and pathogenesis of this disease are unknown, but several hypotheses are discussed. Treatment by a variety of modalities is far from satisfactory, and the prognosis is much poorer than that observed in patients with the "Western" form of intestinal lymphoma. Other differences between Middle Eastern lymphoma and Western lymphoma are described in detail,
本文描述了一名患有中东淋巴瘤的20岁波斯男子，并对文献中另外84例病例进行了回顾。基本上，这种疾病是一种累及上段小肠（十二指肠和空肠近端）的恶性淋巴瘤。它与杵状指、腹痛、体重减轻、腹泻、呕吐和吸收不良有关，并且比“西半球”肠道淋巴瘤更常发生于较年轻的年龄组。一些患者还患有α重链病。性别比例均等，该疾病仅发生于中东和北非的穆斯林和犹太人。上消化道造影常常具有诊断价值，经口小肠活检几乎总能确诊。病理上，中东淋巴瘤具有以下特征：部分或完全绒毛萎缩，表面上皮仅有轻度异常，隐窝稀疏，淋巴管扩张，以及多形性单核细胞浸润固有层并穿透黏膜肌层。本病的病因和发病机制尚不清楚，但讨论了几种假说。多种治疗方式的效果远不尽人意，预后比“西方”型肠道淋巴瘤患者差得多。本文还详细描述了中东淋巴瘤与西方淋巴瘤之间的其他差异。
