Palazzo Giulia
Ematologia, Ospedale S.G. Moscati, Taranto.
Recenti Prog Med. 2020 Mar;111(3):160-166. doi: 10.1701/3315.32858.
Plasma cell leukemia (PCL) is a rare but most aggressive form of monoclonal gammopathies, characterized by the presence of clonal cells in peripheral blood and a poor prognosis. There are two forms of PCL: primary, which arise de novo, and secondary which is a leukemic transformation in patients with previously multiple myeloma. Patients with PCL may benefit from stem cell transplantation and novel agents, but their prognosis remains inferior to that of patients who have multiple myeloma.
We describe the case of 53 years old patient with relapsed plasma cells leukemia after unrelated bone marrow transplant, treated with a KRD chemotherapy regimen. He performed a very good response after the first 2 cycles (bone marrow malignant plasma cells reducing from 36% to 0.5%). However, according to the very poor prognosis of this disease, after the 4th cycle of chemotherapy the patient progressed and dead into few weeks. The KRD regimen was able to convert the chimerism after bone marrow transplant from partial to complete after the first 2 cycles of treatment, showing some activity in this disease.
KRD regimen, in our clinical case, showed some activity being well tolerated in a very poor prognosis disease such as PCL. Probably, right use and maybe sooner use of new drugs such as bortezomib or carfilzomib, in combination regimens, may be useful in better treating such disease.
浆细胞白血病(PCL)是一种罕见但侵袭性极强的单克隆丙种球蛋白病,其特征为外周血中存在克隆细胞且预后较差。PCL有两种形式:原发性,即原发性发生;继发性,是既往患有多发性骨髓瘤患者的白血病转化。PCL患者可能从干细胞移植和新型药物中获益,但其预后仍逊于多发性骨髓瘤患者。
我们描述了一例53岁患者,在接受非亲缘骨髓移植后复发浆细胞白血病,采用KRD化疗方案治疗。在前2个周期后他取得了非常好的反应(骨髓恶性浆细胞从36%降至0.5%)。然而,鉴于该疾病预后极差,在第4周期化疗后患者病情进展并在数周内死亡。KRD方案在治疗的前2个周期后能够将骨髓移植后的嵌合状态从部分转变为完全,显示出对该疾病有一定活性。
在我们的临床病例中,KRD方案显示出一定活性,在如PCL这种预后极差的疾病中耐受性良好。可能,在联合方案中正确使用且或许更早使用硼替佐米或卡非佐米等新药,可能有助于更好地治疗此类疾病。