Liu S Z, Zong X P, Cai W Z, He H J, Ma Z X, Li J Q, Li C X, Wu D P
the First Affiliated Hospital of Soochow University, National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, State Key Laboratory of Radiation Medicine and Protection, Soochow University, Suzhou 215006, China.
Zhonghua Yi Xue Za Zhi. 2024 Jan 2;104(1):69-73. doi: 10.3760/cma.j.cn112137-20230807-00178.
To explore the clinical and pathological characteristics as well as therapies and prognosis of gray zone lymphoma (GZL). The clinical data of 10 GZL patients admitted to the First Affiliated Hospital of Soochow University from December 2016 to December 2022 were retrospectively collected. The clinical and pathological characteristics, therapies and prognosis were analyzed. The cut-off time for follow-up visits was December 31, 2022, and the median time for follow-up visits [(, )] was 40.0 (28.3, 59.8) months. Treatment efficacy was divided into complete remission (CR), partial remission (PR), stable disease (SD) and progressive disease (PD). There were 6 males and 4 females, with a median age [(, )] of 33.5 (27.3-39.5) years. Among them, 8 patients had mediastinal (thymus) involvement and 7 patients were accompanied with extranodal involvement. According to Ann Arbor staging, 1 case was in the limited stage and 9 cases were in the progressive stage. The immunophenotypes of 4 patients were strong expression of CD20, expression of CD30, and no expression of CD15. The immunophenotypes of 6 patients were unequal expression of CD20 and strong expression of CD30 and CD15. One patient received classical hodgkin lymphoma(cHL)-like immunochemotherapy and only achieved PR, and then received enhanced diffuse large b-cell lymphoma (DLBCL)-like immunochemotherapy to achieve CR. Five patients received enhanced DLBCL-like immunochemotherapy for induction therapy and achieved CR. All 4 patients who did not achieve CR achieved CR after receiving second-line or third-line salvage therapy. All patients were given autologous stem cell transplantation (ASCT) for consolidation therapy. One patient relapsed and died during the follow-up visit in the 33rd month, and the remaining patients currently maintained a state of sustained remission. It is found that GZL mostly occurs in young patients, mediastinal involvement is common, and diagnosis relies on pathological morphology and immunophenotype. GZL may be more sensitive to DLBCL-like intensive immune regimens. Sequential ASCT for consolidation can reduce the risk of relapse.
探讨灰色地带淋巴瘤（GZL）的临床及病理特征、治疗方法及预后。回顾性收集2016年12月至2022年12月苏州大学附属第一医院收治的10例GZL患者的临床资料，分析其临床及病理特征、治疗方法及预后。随访截止时间为2022年12月31日，随访时间中位数[（，）]为40.0（28.3，59.8）个月。治疗疗效分为完全缓解（CR）、部分缓解（PR）、病情稳定（SD）和疾病进展（PD）。男性6例，女性4例，年龄中位数[（，）]为33.5（27.3 - 39.5）岁。其中，8例患者有纵隔（胸腺）受累，7例患者伴有结外受累。根据Ann Arbor分期，1例为局限期，9例为进展期。4例患者免疫表型为CD20强表达、CD30表达、CD15不表达；6例患者免疫表型为CD20表达不均、CD30和CD15强表达。1例患者接受经典霍奇金淋巴瘤（cHL）样免疫化疗仅达到PR后，再接受强化弥漫大B细胞淋巴瘤（DLBCL）样免疫化疗达到CR。5例患者接受强化DLBCL样免疫化疗进行诱导治疗并达到CR。4例未达到CR的患者在接受二线或三线挽救治疗后均达到CR。所有患者均接受自体干细胞移植（ASCT）进行巩固治疗。1例患者在随访第33个月时复发死亡，其余患者目前维持持续缓解状态。发现GZL多发生于年轻患者中，纵隔受累常见，诊断依赖病理形态及免疫表型。GZL可能对DLBCL样强化免疫方案更敏感。序贯ASCT进行巩固可降低复发风险。
