实用皮肤病学杂志
實用皮膚病學雜誌
실용피부병학잡지
JOURNAL OF PRACTRCAL DERMATOLOGY
2014年
6期
417-419,422
,共4页
胡治丽%任芳%杨瑞%桑红
鬍治麗%任芳%楊瑞%桑紅
호치려%임방%양서%상홍
淋巴瘤,T细胞,皮下脂膜%临床特征%嗜血细胞综合征%回顾性分析
淋巴瘤,T細胞,皮下脂膜%臨床特徵%嗜血細胞綜閤徵%迴顧性分析
림파류,T세포,피하지막%림상특정%기혈세포종합정%회고성분석
Lymphoma,T-cell,subcutaneous panniculitis-like%Clinical characteristics%HPS%Retrospective analysis
目的:本研究旨在分析皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)的临床特点、治疗及相关的预后因素。方法计算机检索中国期刊全文数据库(CNKI、万方数据库),检索年限1997—2014年,检索词包括“皮下脂膜炎”、“T细胞淋巴瘤”、“淋巴瘤”,共收纳192例SPTCL患者。对这些患者的临床特征,治疗经过,随访结果进行分析。结果192例患者中男97例,女95例,男女发病无差异。发病年龄1岁5个月~82岁,平均年龄34岁。首发部位以四肢(54%),躯干(36%)最常见。肿瘤细胞表达T细胞抗原:CD3、CD45、CD45RO。81例患者就诊初期即出现嗜血细胞综合征(hemophagocytic syndrome,HPS)。92例患者有明确的随访时间,其中位生存时间为9个月,并发HPS患者为43例,未并发HPS患者49例,这两组患者生存率相比差异有统计学意义(P=0.001)。有B症状(发热、乏力、体重下降)的患者与无B症状的患者生存率相比差异有统计学意义(P=0.006)。结论 SPTCL有其特殊的临床特征,诊断主要依靠组织病理检查,免疫组化及基因重排;并发HPS及有全身症状的患者预后较差。
目的:本研究旨在分析皮下脂膜炎樣T細胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)的臨床特點、治療及相關的預後因素。方法計算機檢索中國期刊全文數據庫(CNKI、萬方數據庫),檢索年限1997—2014年,檢索詞包括“皮下脂膜炎”、“T細胞淋巴瘤”、“淋巴瘤”,共收納192例SPTCL患者。對這些患者的臨床特徵,治療經過,隨訪結果進行分析。結果192例患者中男97例,女95例,男女髮病無差異。髮病年齡1歲5箇月~82歲,平均年齡34歲。首髮部位以四肢(54%),軀榦(36%)最常見。腫瘤細胞錶達T細胞抗原:CD3、CD45、CD45RO。81例患者就診初期即齣現嗜血細胞綜閤徵(hemophagocytic syndrome,HPS)。92例患者有明確的隨訪時間,其中位生存時間為9箇月,併髮HPS患者為43例,未併髮HPS患者49例,這兩組患者生存率相比差異有統計學意義(P=0.001)。有B癥狀(髮熱、乏力、體重下降)的患者與無B癥狀的患者生存率相比差異有統計學意義(P=0.006)。結論 SPTCL有其特殊的臨床特徵,診斷主要依靠組織病理檢查,免疫組化及基因重排;併髮HPS及有全身癥狀的患者預後較差。
목적:본연구지재분석피하지막염양T세포림파류(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)적림상특점、치료급상관적예후인소。방법계산궤검색중국기간전문수거고(CNKI、만방수거고),검색년한1997—2014년,검색사포괄“피하지막염”、“T세포림파류”、“림파류”,공수납192례SPTCL환자。대저사환자적림상특정,치료경과,수방결과진행분석。결과192례환자중남97례,녀95례,남녀발병무차이。발병년령1세5개월~82세,평균년령34세。수발부위이사지(54%),구간(36%)최상견。종류세포표체T세포항원:CD3、CD45、CD45RO。81례환자취진초기즉출현기혈세포종합정(hemophagocytic syndrome,HPS)。92례환자유명학적수방시간,기중위생존시간위9개월,병발HPS환자위43례,미병발HPS환자49례,저량조환자생존솔상비차이유통계학의의(P=0.001)。유B증상(발열、핍력、체중하강)적환자여무B증상적환자생존솔상비차이유통계학의의(P=0.006)。결론 SPTCL유기특수적림상특정,진단주요의고조직병리검사,면역조화급기인중배;병발HPS급유전신증상적환자예후교차。
ObjectiveTo analyze the clinical characteristics, treatment and prognosis related factors of subcutaneous panniculitis like T cell lymphoma (SPTCL).MethodsTotal 192 patients of SPTCL reported in the literatures in China were included by searching Wanfang and CNKI databases with the keywords of "subcutaneous panniculitis", "T-cell lymphoma" and "lymphoma", then the clinical characteristics, treatments and follow-up results were analyzed.ResultsThere were roughly equal numbers of male and female patients reported (n=95 andn=97, respectively). The median age at diagnosis was 34 years (range, 1.5-82years). The skin lesions predominantly affect the extremities (54%) and trunk (36%), but can also occur on face and buttocks. Most neoplastic lymphocytes expressed CD3, CD45 and CD45RO. At the time of diagnosis, hemophagocytic syndrome (HPS) was reported in 81 of 192 patients (42%). The presence or absence of HPS, as well as the presence or absence of B symptoms may led to signiifcant difference in prognosis (P=0.001 andP=0.006, respectively).ConclusionSPTCL is a rare subtype of primary cutaneous lymphoma which has unique clinical features. The presence of HPS or B symptoms may indicate a poor prognosis.