Yang Yang, Fu Li-Juan, Chen Chun-Mei, Hu Mei-Wei
Department of Hematology, The Second Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310014, Zhejiang Province, China.
World J Clin Cases. 2021 Feb 16;9(5):1175-1183. doi: 10.12998/wjcc.v9.i5.1175.
Conventional therapies for primary plasma cell leukemia (pPCL) are usually ineffective, with a short remission time with the use of multiple myeloma medications, showing aggressiveness of pPCL. B-cell lymphoma-2 inhibitor venetoclax is usually used for relapsed/refractory multiple myeloma (RRMM) with t(11;14). There are very few studies published on the use of venetoclax in pPCL without t(11;14). Similarly, histone deacetylase inhibitors are considered effective for the treatment of RRMM, but there are no reports on their use in pPCL.
A 57-year-old woman with severe anemia, thrombocytopenia, multiple bone destruction, impaired renal function, and 42.7% of peripheral plasma cells is reported. After multiple chemotherapy regimens and chimeric antigen receptor T-cell treatment, the disease progressed again. The patient had very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.
The success of venetoclax-chidamide-dexamethasone combination therapy in achieving a very good partial response suggested that it can be used for refractory/relapsed pPCL patients who have been exhausted with the use of various drug combinations and had poor survival outcomes.
原发性浆细胞白血病(pPCL)的传统治疗通常无效,使用多种骨髓瘤药物时缓解时间较短,显示出pPCL的侵袭性。B细胞淋巴瘤-2抑制剂维奈克拉通常用于治疗伴有t(11;14)的复发/难治性多发性骨髓瘤(RRMM)。关于在无t(11;14)的pPCL中使用维奈克拉的研究报道极少。同样,组蛋白去乙酰化酶抑制剂被认为对RRMM治疗有效,但尚无其在pPCL中应用的报道。
报告了一名57岁女性,患有严重贫血、血小板减少、多处骨质破坏、肾功能受损,外周血浆细胞占42.7%。在接受多种化疗方案和嵌合抗原受体T细胞治疗后,疾病再次进展。该患者获得了非常好的部分缓解,并通过维奈克拉联合西达本胺和地塞米松治疗维持了较长时间。
维奈克拉-西达本胺-地塞米松联合治疗成功实现了非常好的部分缓解,表明其可用于因使用各种药物组合而治疗无效且生存预后较差的难治性/复发性pPCL患者。