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Small intestinal presentation of nodular lymphocyte-predominant Hodgkin lymphoma with T cell/histiocyte-rich B cell lymphoma-like areas—with review of literature on extranodal presentation of this disease

机译:结节性淋巴细胞为主的霍奇金淋巴瘤的小肠表现,并伴有T细胞/富含组织细胞的B细胞淋巴瘤样区域-对该疾病结外表现的文献进行综述

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摘要

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), accounts for ∼5% of all cases of Hodgkin lymphoma and is characterized by involvement of the peripheral lymph nodes. NLPHL occurs in young adults and is associated with frequent relapses. In 3% to 7% of cases, NLPHL progresses to a diffuse large B cell lymphoma. Furthermore, a proportion of NLPHL also have areas with features of T cell/histiocyte-rich large B cell lymphoma (THRLBCL), either at presentation or on follow-up. Here, we describe a 32-year-old man who presented to the emergency department with small bowel perforation. The resected small bowel showed full-thickness mural ulceration and involvement by a lymphoma with features of NLPHL that also had areas resembling THRLBCL. The patient had axillary lymphadenopathy, biopsy of which showed NLPHL with focal THRLBCL-like areas. Such a lymphoma presenting as small intestinal lesion/perforation has not been reported in the literature before. We take this opportunity to review the literature on extranodal presentations of NLPHL and discuss the natural history of this disease.
机译:结节性淋巴细胞为主的霍奇金淋巴瘤(NLPHL)约占所有霍奇金淋巴瘤病例的5%,其特征是周围淋巴结受累。 NLPHL发生在年轻人中,并与复发频繁相关。在3%至7%的病例中,NLPHL会进展为弥漫性大B细胞淋巴瘤。此外,在报告时或随访时,一定比例的NLPHL还具有具有T细胞/组织细胞丰富的大B细胞淋巴瘤(THRLBCL)特征的区域。在这里,我们描述了一个32岁的男子,他带着小肠穿孔出现在急诊室。切除的小肠显示全层壁溃疡,并被淋巴瘤累及,淋巴瘤的特征为NLPHL,其区域也类似THRLBCL。该患者患有腋窝淋巴结肿大,活检显示为NLPHL并伴有局灶性THRLBCL样区域。以前没有文献报道过这种表现为小肠病变/穿孔的淋巴瘤。我们借此机会回顾了有关NLPHL结外表现的文献,并讨论了这种疾病的自然史。

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