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Review
. 2023 Mar 14;44(3):242-246.
doi: 10.3760/cma.j.issn.0253-2727.2023.03.011.

[Gray zone lymphoma: five cases report and literature review]

[Article in Chinese]
H S Zou  1 , H J Zhang  1 , H M Liu  1 , W Y Huang  1 , W Liu  1 , R Lyu  1 , T Y Wang  1 , W W Sui  1 , M W Fu  1 , Q Wang  1 , L G Qiu  1 , D H Zou  1
Affiliations
Review

[Gray zone lymphoma: five cases report and literature review]

[Article in Chinese]
H S Zou et al. Zhonghua Xue Ye Xue Za Zhi. .

Abstract
in English, Chinese

Objective: To investigate the clinical and pathological features, treatment, and prognosis of gray zone lymphoma (GZL) . Methods: 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. Results: 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. Conclusions: 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.

目的： 探索灰区淋巴瘤（GZL）的临床及病理特征、治疗及预后。 方法： 回顾性分析2013年7月2日至2021年2月10日就诊于中国医学科学院血液病医院的5例灰区淋巴瘤患者的临床表现、病理学特征、治疗及转归情况。 结果： 男1例，女4例，中位年龄28（16~51）岁。4例患者纵隔（胸腺）受累，其中2例伴上腔静脉阻塞综合征，3例伴结外受累。Ann Arbor分期局限期1例，进展期4例。3例患者的病理形态与经典型霍奇金淋巴瘤（cHL）类似，可见霍奇金样细胞散在分布，免疫表型为CD20强表达，CD30表达而CD15不表达；另2例患者形态兼具cHL和弥漫大B细胞淋巴瘤（DLBCL）特征，部分区域形似霍奇金细胞，部分区域则形似免疫母细胞，免疫表型CD20表达强弱不等，CD30和CD15强表达。2例患者采用cHL样方案诱导治疗，仅获得部分缓解；予DLBCL样增强免疫化疗方案挽救治疗后均获得完全缓解（CR）。3例患者给予增强的DLBCL样免疫化疗方案诱导治疗，2例患者有效，其中1例获得CR。4例未获得CR的患者接受二线或三线挽救治疗后均获得CR，其中3例给予自体造血干细胞移植（ASCT）巩固治疗。1例患者失访，1例患者在诊断后35.9个月时因疾病进展死亡，其余3例患者均维持持续缓解状态。 结论： GZL罕见，多发生于年轻患者，纵隔受累常见，诊断依赖病理形态和免疫表型。初诊GZL可能对DLBCL样强化免疫方案更敏感，复发难治患者可考虑非交叉耐药的联合化疗或联合新药。.

Keywords: Drug therapy, combination; Lymphoma, B-cell; Mediastinal.

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Conflict of interest statement

利益冲突 所有作者声明无利益冲突

Figures

图1
图1. 例5颈部淋巴结病理组织学形态及免疫表型 A 颈部淋巴结HE染色，低倍镜下可见纤维组织增生和灶性坏死（×7）；B 肿瘤细胞呈片状增生（HE染色，×200）；C 肿瘤细胞形态多样，部分形态似免疫母细胞，部分形态似霍奇金细胞，可见双核及多核肿瘤细胞（HE染色，×400）；D 肿瘤细胞CD30阳性（×200）；E 肿瘤细胞CD15阳性（×200）；F 肿瘤细胞CD20异质性表达（×200）
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