Zou H S, Zhang H J, Liu H M, Huang W Y, Liu W, Lyu R, Wang T Y, Sui W W, Fu M W, Wang Q, Qiu L G, Zou D H
Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2023 Mar 14;44(3):242-246. doi: 10.3760/cma.j.issn.0253-2727.2023.03.011.
To investigate the clinical and pathological features, treatment, and prognosis of gray zone lymphoma (GZL) . From July 2, 2013, to February 10, 2021, the clinical and pathological features, treatment, and outcomes of five patients with GZL at the Blood Diseases Hospital, Chinese Academy of Medical Sciences were studied retrospectively. There were one male and 4 females, with a median age of 28 (16-51) years at diagnosis. Four patients had mediastinal (thymic) involvement, two of which had superior vena cava obstruction syndrome, and 3 patients had extra-nodal involvement. There was one case with a limited Ann Arbor stage and 4 cases with a progressive stage. Three patients had cHL-like pathomorphology with scattered Hodgkin-like cells, strongly positive for CD20, positive for CD30, and CD15 was negative; the other two patients had both cHL and DLBCL morphology, with some areas resembling Hodgkin cells and some areas resembling immunoblasts, strongly positive for CD30, and CD15 but negative CD20. Two patients were treated with cHL-like regimens for induction and achieved only partial remission; after salvage therapy with enhanced DLBCL-like regimens, all achieved complete remission (CR) . Three patients were treated with enhanced DLBCL-like immunochemotherapy regimens for induction, and two patients were effective, one of whom achieved CR. Four patients who did not achieve CR were given second or third-line salvage therapy, and all of them recovered. One patient lost parity, one died of disease progression at 35.9 months after diagnosis, and the remaining three maintained sustained remission. GZL is uncommon, usually affects younger patients, is mediastinal and is diagnosed using path morphology and immunophenotype. Patients with newly diagnosed GZL appear to be more sensitive to DLBCL-like immunochemotherapy regimens; relapsed or refractory patients were tended with non-cross-resistant combination chemotherapy or with new drugs.
探讨灰色地带淋巴瘤(GZL)的临床病理特征、治疗方法及预后。回顾性研究2013年7月2日至2021年2月10日在中国医学科学院血液病医院确诊的5例GZL患者的临床病理特征、治疗方法及治疗结果。患者中男性1例,女性4例,确诊时中位年龄为28岁(16 - 51岁)。4例患者有纵隔(胸腺)受累,其中2例有上腔静脉阻塞综合征,3例有结外受累。Ann Arbor分期为局限期1例,进展期4例。3例患者具有经典型霍奇金淋巴瘤(cHL)样病理形态,散在霍奇金样细胞,CD20强阳性,CD30阳性,CD15阴性;另外2例患者同时具有cHL和弥漫大B细胞淋巴瘤(DLBCL)形态,部分区域类似霍奇金细胞,部分区域类似免疫母细胞,CD30强阳性,CD15阳性但CD20阴性。2例患者采用cHL样方案诱导治疗,仅获得部分缓解;采用强化的DLBCL样方案挽救治疗后,均获得完全缓解(CR)。3例患者采用强化的DLBCL样免疫化疗方案诱导治疗,2例有效,其中1例获得CR。4例未达到CR的患者接受二线或三线挽救治疗,均康复。1例患者病情进展,1例在诊断后35.9个月死于疾病进展,其余3例维持持续缓解。GZL较为罕见,通常累及年轻患者,多位于纵隔,通过病理形态和免疫表型进行诊断。初诊的GZL患者似乎对DLBCL样免疫化疗方案更敏感;复发或难治性患者倾向于采用非交叉耐药的联合化疗或新药治疗。