{"title":"原发性皮肤CD8+侵袭性表皮细胞毒性t细胞淋巴瘤临床分析","authors":"Ping Cheng, Jun Guan, Yan Feng, Hui Cheng","doi":"10.19746/j.cnki.issn.1009-2137.2025.03.022","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8<sup>+</sup> aggressive epidermotropic cytotoxic T-cell lymphoma (CD8<sup>+</sup> PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma.</p><p><strong>Methods: </strong>The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8<sup>+</sup> PCAECTL admitted to our hospital were retrospectively analyzed.</p><p><strong>Results: </strong>The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8<sup>+</sup> PCAECTL, T3aN<sub>0</sub>M<sub>0</sub> stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m<sup>2</sup>) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m<sup>2</sup>, d 1 + cytarabine 2 g/m<sup>2</sup>, q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34<sup>+</sup> cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT.</p><p><strong>Conclusion: </strong>CD8<sup>+</sup> PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8<sup>+</sup> cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8<sup>+</sup> PCAECTL patients.</p>","PeriodicalId":35777,"journal":{"name":"中国实验血液学杂志","volume":"33 3","pages":"777-783"},"PeriodicalIF":0.0000,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Clinical Analysis of Primary Cutaneous CD8<sup>+</sup> Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma].\",\"authors\":\"Ping Cheng, Jun Guan, Yan Feng, Hui Cheng\",\"doi\":\"10.19746/j.cnki.issn.1009-2137.2025.03.022\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8<sup>+</sup> aggressive epidermotropic cytotoxic T-cell lymphoma (CD8<sup>+</sup> PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma.</p><p><strong>Methods: </strong>The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8<sup>+</sup> PCAECTL admitted to our hospital were retrospectively analyzed.</p><p><strong>Results: </strong>The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8<sup>+</sup> PCAECTL, T3aN<sub>0</sub>M<sub>0</sub> stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m<sup>2</sup>) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m<sup>2</sup>, d 1 + cytarabine 2 g/m<sup>2</sup>, q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34<sup>+</sup> cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT.</p><p><strong>Conclusion: </strong>CD8<sup>+</sup> PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8<sup>+</sup> cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8<sup>+</sup> PCAECTL patients.</p>\",\"PeriodicalId\":35777,\"journal\":{\"name\":\"中国实验血液学杂志\",\"volume\":\"33 3\",\"pages\":\"777-783\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"中国实验血液学杂志\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.19746/j.cnki.issn.1009-2137.2025.03.022\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"中国实验血液学杂志","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.19746/j.cnki.issn.1009-2137.2025.03.022","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
目的:报道1例原发性皮肤CD8+侵袭性表皮细胞毒性t细胞淋巴瘤(CD8+ PCAECTL)的临床特点、诊断、治疗及预后,提高对这种极为罕见的淋巴瘤的认识。方法:回顾性分析我院收治的1例CD8+ PCAECTL患者的临床表现、诊断、病程及预后。结果:患者女,42岁,以鼻、面部及背部浸润性皮疹为主要临床表现。经病变皮肤组织病理、免疫组织化学、分子生物学及影像学检查,确诊为CD8+ PCAECTL, T3aN0M0期。交替化疗CHOP/HD-MTX(甲氨蝶呤,6 g/m2)方案,4个周期后完全缓解(CR)。经dhp方案化疗(顺铂100 mg/m2, d 1 +阿糖胞苷2 g/m2, q 12h, d 2 +地塞米松40 mg/d, d 1-4)后,利用重组人粒细胞集落刺激因子(g - csf)动员患者外周血干细胞,成功收集足够数量的CD34+细胞。采用BEAM方案进行预处理,然后采用自体造血干细胞移植(AHSCT)进行巩固治疗。患者在ahsct后随访20个月后仍处于无病生存状态。结论:CD8+ PCAECTL在临床上极为罕见,起病隐匿,早期诊断困难。主要表现为表皮性CD8+细胞毒性T细胞增生,临床病程具有侵袭性。目前尚没有统一的最佳治疗方案标准,预后很差。诱导化疗缓解后AHSCT巩固治疗可改善CD8+ PCAECTL患者的生存和预后。
Objective: To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8+ PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma.
Methods: The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8+ PCAECTL admitted to our hospital were retrospectively analyzed.
Results: The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8+ PCAECTL, T3aN0M0 stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m2) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m2, d 1 + cytarabine 2 g/m2, q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34+ cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT.
Conclusion: CD8+ PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8+ cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8+ PCAECTL patients.