Fan Zhi-Min, Wu Dao-Lei, Xu Neng-Wen, Ye Li, Yan Li-Ping, Li Lin-Jie, Zhang Jun-Yu
Department of Hematology, Lishui Municipal Central Hospital, Lishui 323000, Zhejiang Province, China.
Department of Ophthalmology, Lishui Municipal Central Hospital, Lishui 323000, Zhejiang Province, China.
World J Clin Cases. 2024 May 26;12(15):2655-2663. doi: 10.12998/wjcc.v12.i15.2655.
High-grade B-cell lymphoma (HGBL) is an unusual malignancy that includes myelocytomatosis viral oncogene (), B-cell lymphoma-2 (), and/or rearrangements, termed double-hit or triple-hit lymphomas, and HGBL-not otherwise specific (HGBL-NOS), which are morphologically characteristic of HGBL but lack , , or rearrangements. HGBL is partially transformed by follicular lymphoma and other indolent lymphoma, with few cases of marginal zone lymphoma (MZL) transformation. HGBL often has a poor prognosis and intensive therapy is currently mainly advocated, but there is no good treatment for these patients who cannot tolerate chemotherapy.
We reported a case of MZL transformed into HGBL-NOS with mutation and terminal deoxynucleotidyl transferase expression. Gene analysis revealed the gene expression profile was identical in the pre- and post-transformed tissues, suggesting that the two diseases are homologous, not secondary tumors. The chemotherapy was ineffective and the side effect was severe, so we tried combination therapy including venetoclax and obinutuzumab. The patient tolerated treatment well, and reached partial response. The patient had recurrence of hepatocellular carcinoma and died of multifunctional organ failure. He survived for 12 months after diagnosis.
Venetoclax combined with obinutuzumab might improve the survival in some HGBL patients, who are unsuitable for chemotherapy.
高级别B细胞淋巴瘤（HGBL）是一种特殊的恶性肿瘤，包括髓细胞瘤病毒癌基因（）、B细胞淋巴瘤-2（）和/或重排，称为双打击或三打击淋巴瘤，以及高级别B细胞淋巴瘤，非特指型（HGBL-NOS），其在形态学上具有HGBL的特征，但缺乏、或重排。HGBL部分由滤泡性淋巴瘤和其他惰性淋巴瘤转化而来，边缘区淋巴瘤（MZL）转化的病例较少。HGBL的预后通常较差，目前主要提倡强化治疗，但对于这些无法耐受化疗的患者没有好的治疗方法。
我们报告了1例MZL转化为HGBL-NOS并伴有突变和末端脱氧核苷酸转移酶表达的病例。基因分析显示转化前后组织的基因表达谱相同，提示这两种疾病是同源的，而非继发性肿瘤。化疗无效且副作用严重，因此我们尝试了包括维奈克拉和奥妥珠单抗在内的联合治疗。患者对治疗耐受性良好，达到部分缓解。患者出现肝细胞癌复发，死于多器官功能衰竭。确诊后存活12个月。
维奈克拉联合奥妥珠单抗可能会改善一些不适合化疗的HGBL患者的生存情况。
