Single-Center Experience of Patients with Plasma Cell Leukemia in the Era of New Therapeutics.

IF 1.7 4区 医学 Q3 HEMATOLOGY Acta Haematologica Pub Date : 2024-05-22 DOI:10.1159/000539223
Maria Dampmann, Sarah Flossdorf, Julius Keyl, Hans Christian Reinhardt, Christine Hanoun
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Abstract

Introduction: Plasma cell leukemia (PCL) can occur de novo as primary PCL (pPCL), or in patients with prior diagnosis of multiple myeloma (MM) as secondary PCL (sPCL). In 2021, the diagnostic criteria have been revised, establishing a new cut-off of ≥5% plasma cells in the peripheral blood. Lacking specific clinical trials, PCL is treated similarly to MM; however, outcome for patients with PCL remains poor. Here, we report outcomes for patients with pPCL and sPCL in the era of novel agents.

Methods: We performed a retrospective analysis and identified 19 patients (11 pPCL, 8 sPCL) who have been treated for PCL between 2010 and 2022 at University Hospital Essen.

Results: Patients with pPCL had a median overall survival (OS) of 37.8 months (95% CI: [15.4; 52.3] months) from diagnosis, with a median time to next treatment (TTNT) of 18.4 (2.0; 22.9) months. All patients were treated with a proteasome-inhibitor (PI)-based induction therapy, and the majority was consolidated with an autologous stem cell transplantation (SCT). Five of these patients received a tandem transplantation. Patients with sPCL had a median OS of only 1.5 months after diagnosis of PCL. Only 1 patient achieved a remission with daratumumab and subsequent allogenic SCT.

Conclusion: With our study, we add evidence for a PI-based induction therapy followed by a consolidating autologous SCT for patients with pPCL and give further evidence that a tandem transplant concept might be beneficial. The diagnosis of sPCL remains devastating and needs new therapeutic approaches.

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新疗法时代浆细胞白血病患者的单中心经验。
导言:浆细胞白血病(PCL)可作为原发性 PCL(pPCL)从新发生,也可作为继发性 PCL(sPCL)发生于既往诊断为多发性骨髓瘤(MM)的患者。2021 年,对诊断标准进行了修订,确定了外周血中浆细胞含量≥ 5% 的新临界值。由于缺乏具体的临床试验,PCL的治疗方法与MM类似,但PCL患者的预后仍然不佳。在此,我们报告了新型药物时代 pPCL 和 sPCL 患者的治疗效果:我们进行了一项回顾性分析,确定了 2010 年至 2022 年期间埃森大学医院收治的 19 例 PCL 患者(11 例 pPCL,8 例 sPCL):pPCL患者自确诊起的中位总生存期(OS)为37.8个月(95% CI:[15.4; 52.3]个月),中位下次治疗时间(TTNT)为18.4 [2.0; 22.9]个月。所有患者都接受了以蛋白酶体抑制剂(PI)为基础的诱导治疗,大多数患者接受了自体干细胞移植(SCT)巩固治疗。其中五名患者接受了串联移植。sPCL患者在确诊PCL后的中位OS仅为1.5个月。只有一名患者通过达拉单抗和随后的异基因SCT获得了缓解:通过我们的研究,我们为pPCL患者采用基于PI的诱导疗法,然后进行巩固性自体干细胞移植增加了证据,并进一步证明串联移植概念可能是有益的。sPCL的诊断仍然令人沮丧,需要新的治疗方法。
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来源期刊
Acta Haematologica
Acta Haematologica 医学-血液学
CiteScore
4.90
自引率
0.00%
发文量
61
审稿时长
6-12 weeks
期刊介绍: ''Acta Haematologica'' is a well-established and internationally recognized clinically-oriented journal featuring balanced, wide-ranging coverage of current hematology research. A wealth of information on such problems as anemia, leukemia, lymphoma, multiple myeloma, hereditary disorders, blood coagulation, growth factors, hematopoiesis and differentiation is contained in first-rate basic and clinical papers some of which are accompanied by editorial comments by eminent experts. These are supplemented by short state-of-the-art communications, reviews and correspondence as well as occasional special issues devoted to ‘hot topics’ in hematology. These will keep the practicing hematologist well informed of the new developments in the field.
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