张明智, 邱亚娟, 李文才, 王冠男, 李玲. 6例皮下脂膜炎样T细胞淋巴瘤临床病理特征及治疗分析[J]. 中国肿瘤临床, 2011, 38(18): 1111-1113. DOI: 10.3969/j.issn.1000-8179.2011.18.014
引用本文: 张明智, 邱亚娟, 李文才, 王冠男, 李玲. 6例皮下脂膜炎样T细胞淋巴瘤临床病理特征及治疗分析[J]. 中国肿瘤临床, 2011, 38(18): 1111-1113. DOI: 10.3969/j.issn.1000-8179.2011.18.014
Mingzhi ZHANG, Yajuan QIU, Wencai LI, Guannan WANG, Ling LI. Analysis of Six Cases of Subcutaneous Panniculitis-like T-cell Lymphoma[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2011, 38(18): 1111-1113. DOI: 10.3969/j.issn.1000-8179.2011.18.014
Citation: Mingzhi ZHANG, Yajuan QIU, Wencai LI, Guannan WANG, Ling LI. Analysis of Six Cases of Subcutaneous Panniculitis-like T-cell Lymphoma[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2011, 38(18): 1111-1113. DOI: 10.3969/j.issn.1000-8179.2011.18.014

6例皮下脂膜炎样T细胞淋巴瘤临床病理特征及治疗分析

Analysis of Six Cases of Subcutaneous Panniculitis-like T-cell Lymphoma

  • 摘要: 观察皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床特点、病理及免疫表型,探讨其有效的治疗方法及预后因素。方法:回顾性分析6例SPTCL患者临床、病理特征及治疗疗效。结果:6例患者均表现为不同部位皮下结节和(或)硬结性红斑,伴或不伴淋巴结肿大。其中2例侵犯骨髓,2例EBV阳性,5例伴有发热。所有患者均有典型的病理学和免疫表型改变,肿瘤细胞浸润皮下小叶及脂肪组织,镜下淋巴瘤细胞花环状围绕单个脂肪细胞排列,中等或大细胞,染色质浓染。细胞表达T细胞标记物CD3及CD45RO,不表达B细胞标志物CD20及CD79a,1例CD56阳性,3例TIA-1阳性,4例GranzymeB阳性,3例初始治疗应用CHOP均效果差未达到持续缓解,应用含吉西他滨方案治疗初治和复发患者4例,3例完全缓解(CR),1例部分缓解(PR),均未出现严重血液学毒性。1例合并噬血细胞综合征(HPS)和EBV感染者死亡。结论:含吉西他滨的联合化疗方案是一种对初治及复发SPTCL均有效的治疗方法。伴HPS、合并EBV感染是其不良预后因素。

     

    Abstract: To investigate the clinical, pathological, and immunophenotypic characteristics of subcutaneous panniculitis-like T cell lymphoma and to investigate the corresponding effective treatment and prognostic factors. Methods: The clinical, histologic, immunophenotypic features and treatments of 6 cases were described in detail. Results: All 6 patients presented with subcutaneous nodules and/or erythematous plaques with or without lymphadenopathy, and 2 cases had bone marrow involvement, 2 cases were positive for Epstein-Barr virus, and 5 cases had fever. All 6 patients presented with typical histologic changes. Intermediate to large-sized lymphocytes infiltrated preferentially into the lobular area of the subcutaneous tissue and displayed adipotropism with rimming of lymphocytes around adipocytes and empty lipid vacuoles with hyperchromatic nuclei. T cell markers were positive, B cell markers were negative; CD4 and CD8 expression were different. The cytotoxic proteins in some cases were positive. The three patients treated with CHOP all relapsed. Chemotherapy with gemcitabine for newly diagnosed and 4 relapsed patients achieved complete remission ( CR ) in 3 cases, partial remission ( PR ) in 1 case with no severe hematologic toxicity. One case with hemophagocytic syndrome ( HPS ) that was Epstein-Barr virus-positive died. Conclusion: Chemotherapy with gemcitabine is an effective regimen in the treatment of newly diagnosed and relapsed SPTCL patients. The presence of HPS and Epstein-Barr virus are the poor prognostic factors.

     

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