酷似白塞病的淋巴瘤八例分析并文献复习

Lymphoma mimicking Behcet＇s disease： case report and literature review

目的探讨酷似白塞病的淋巴瘤患者的特征及转归，以减少临床误诊。方法回顾性分析2015年我院收治的2例酷似白塞病的淋巴瘤患者，并检索中英文文献库中误诊为白塞病的淋巴瘤患者6例，分析其临床特点。结果8例患者中6例为非霍奇金淋巴瘤；7例有口腔溃疡，其中2fflJ溃疡位于上颚；6例有外阴溃疡；所有患者均存在皮肤病变，表现为痤疮或者脓疱疹；4例有眼炎；2例针刺试验阳性；7例发热，其中6例为中高热，4例为高热，1例为低热。4例出现白细胞减少，4例存在淋巴细胞减低。上述患者均符合2014年白塞病国际评分标准，分值为4-8分，平均（5．8±1．5）分。患者生存期1～36个月，中位生存期8个月。结论对于疑似白塞病的患者，中高度发热、不典型部位黏膜溃疡、外周血白细胞及淋巴细胞减少应警惕淋巴瘤。

Objective To study the clinical characteristics and outcome of two lymphoma patients mimicking Behcet＇s disease. Methods Lymphoma was diagnosed in two patients mimicking Behcet＇s disease referred to our Department in 2015. A search on published similar cases in Chinese database and the Pubmed was also performed and then analyzed. Results Eight patients were indentified in this pooled analysis, six of which were non-Hodgkin lymphoma （NHL）. All of the eight cases presented with cutaneous lesion, seven cases with fever, seven cases with oral ulceration and six cases with orogenital ulceration, respectively. Ocular involvement was present in four of the eight cases, two were with a positive pathergy test. Among feverish patients, six were moderate or high fever, four were high fever, one was low-grade fever. Neutropenia was found in four patients, and lymphocytoponia in four of five patients with detailed data. All patients fulfilled the 2014 International Criteria for Behcet＇s Disease （ICBD） with an average score of （5.8±1.5）, ranging from 4 to 8. Survival period ranged from one month to 36 months, with an average of 8 months. Conclusion For patients diagnosed as Behcet＇s disease are finally diagnosed as lymphoma. For patients with Behcet＇s disease present- ation but also present with mediate to high fever, atypical deepseated ulcer, neutropenia or lymphocytoponia, malignancy especially lymphoma should be investigated.