Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Eur J Haematol. 2018 Feb;100(2):215-217. doi: 10.1111/ejh.12986. Epub 2017 Nov 16.
Primary plasma cell leukemia (pPCL) is the most aggressive form of the plasma cell (PC) malignancy, multiple myeloma (MM). It has been commonly associated with the presence of a chromosome translocation involving the immunoglobulin heavy chain (IgH) locus on 14q32, that is t (11;14). Results from early phase clinical trials utilizing the selective Bcl-2 inhibitor, venetoclax, as a single agent in patients with relapsed MM have had remarkable efficacy among patients with t (11;14) abnormality. The present case demonstrates the ability of a combination regimen incorporating Bcl-2 inhibition with daratumumab, bortezomib, venetoclax, and dexamethasone to induce a rapid and very deep hematologic response in a pPCL patient with t (11;14), even in a setting of very refractory disease. This case highlights the need to further study Bcl-2 inhibition-based therapy as an option for therapy in patients with pPCL with t (11;14).
原发性浆细胞白血病（pPCL）是浆细胞（PC）恶性肿瘤多发性骨髓瘤（MM）中最具侵袭性的形式。它通常与涉及 14q32 上免疫球蛋白重链（IgH）基因座的染色体易位有关，即 t（11；14）。早期临床试验的结果表明，在复发 MM 患者中，作为单一药物使用选择性 Bcl-2 抑制剂维奈托克（venetoclax），在 t（11；14）异常患者中具有显著疗效。本病例证明了包含 Bcl-2 抑制、达雷妥尤单抗、硼替佐米、维奈托克和地塞米松的联合方案能够诱导 t（11；14）的 pPCL 患者快速且深度的血液学缓解，即使在疾病非常难治的情况下也是如此。该病例强调了需要进一步研究基于 Bcl-2 抑制的治疗作为 t（11；14）的 pPCL 患者治疗选择的必要性。
