误诊为风湿病的皮下脂膜炎样T细胞淋巴瘤5例临床分析

Clinical analysis of five cases of subcutaneous panniculitis like T-cell lymphoma misdiagnosed as rheumatic diseases

目的探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床表现及病理特点,以提高SPTCL的诊断水平。方法对2005年1月～2012年1月该科收治的5例SPTCL患者的临床、病理特点进行总结和分析。结果5例患者中,男性2例,女性3例,中位发病年龄23.8岁(14～38岁)。发病至确诊时间平均8个月(3～17个月),初诊均考虑为风湿病(多发性肌炎、结节性脂膜炎、抗磷脂综合征),主要临床表现为多发皮下结节、肿块,可累及四肢、躯干及头面部。2例发生破溃,1例侵犯乳腺,2例侵犯淋巴结。5例均伴有发热、肝功能损伤及LDH升高,4例外周血白细胞减少,2例患者出现嗜血细胞综合征(HPS)。病理显示异型淋巴细胞浸润皮下脂肪,呈脂膜炎样分布。5例瘤细胞均表达CD45RO、CD3,不表达CD20。2例经化疗分别随访21个月及3个月,1例死亡,1例存活,3例失访。结论 SPTCL临床表现为反复皮下结节,容易误诊为脂膜炎等风湿病。当患者抗风湿治疗效果不佳,特别是伴LDH明显升高、白细胞减少、甚至出现HPS时,要警惕SPTCL的可能。病理活检行免疫组织化学检测及T细胞受体基因重排PCR检测可明确诊断。

【Objective】 To explore the clinical presentations and pathologic features of the subcutaneous panniculitis like T-cell lymphoma（SPTCL） in order to improve its diagnosis.【Methods】 Five patients treated as SPTCL in our hospital from January 2005 to January 2009 were included in this study,and the clinical, pathologic,and immunohistochemical features were analyzed.【Results】 There were 2 males and 3 females with the median age 23.8 years（range： 14～38 years）.The period from the onset of symptom to the diagnosis as SPTCL was 8 months on average（range： 14～38 years）.The 5 patients had been misdiagnosed as rheumatic diseases at beginning,such as polymyositis,nodular panniculitis,antiphospholipid syndrome.All the patients presented with multiple subcutaneous nodules or deeply seated plaques at different sites,most commonly on the extremities and trunk,as well as on face and neck.Ulceration of nodules occurred in 2 cases.2 patients had lymphadenopathy involvement,1 patient had breast involvement.5 cases all had fevered and abnormal liver function with higher level of lactate dehydrogenase（LDH）.4 cases had smaller amount of white blood cell.2 patients had hemophagocytic syndrome（HPS）,and the pathologic features may consist of infiltrations of atypical lymphocytes,infiltrated subcutaneous lipid tissue as panniculitis pattern.CD3 and CD45RO were expressed in the tumor cells of all 5 cases.CD 20 was not expressed.Loss of the follow-up was in 3 cases, one patient was died,and the other patient was alive with a followed-up of 21 months and 3 months respectively.【Conclusion】 SPTCL is often misdiagnosed as rheumatic disease at initial stage.SPTCL should be watched out once the anti-rheumatic therapy does not work well,especially accompanied by high level LDH in serum,HPS and lower WBC.Immunophenotype and gene rearrangement data are considered important in confirming a diagnosis of SPTCL.