Survival outcomes between haploidentical stem cell transplantation and chemotherapy for blastic plasmacytoid dendritic cell neoplasm

IF 4.5 2区 医学 Q1 HEMATOLOGY
Jian Zhou, Fengrong Wang, Shenmiao Yang, Yuanyuan Zhang, Yang Liu, Ting Zhao, Yuqian Sun, Shen Zhang, Xiaodong Mo, Huan Chen, Pan Suo, Lei Wen, Jinsong Jia, Jing Wang, Robert Peter Gale, Jin Lu
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Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive and rare hematological malignancy with poor clinical outcomes. Stem cell transplantation helps to achieve long-term survival in adults. However, the benefit of haploidentical stem cell transplantation (HID-SCT) versus chemotherapy is unclear with BPDCN. We retrospectively analyzed 32 patients diagnosed with BPDCN including 15 who underwent HID-SCT and 17 who only received chemotherapy. The median age was 52 (range, 19–78) years. The ratio of male/female was 2.2. Skin, bone marrow and lymph node were the most three common sites of disease involvement. Compared with the chemotherapy group, patients in the HID-SCT group had significantly better progression-free survival (PFS; median, 7 months versus not reached, P < 0.001) and overall survival (OS; median, 13 months versus not reached, P < 0.001). The 4-year rates for PFS and OS in transplant patients were 74% (95% Confidence Interval [CI], 47, 100%) and 93% (79, 100%), respectively, compared to 0 in non-transplant patients. In conclusion, our results demonstrated HID-SCT could provide long-term remissions in BPDCN patients.

Abstract Image

单倍体干细胞移植和化疗治疗母浆细胞样树突状细胞肿瘤的生存结果。
母浆细胞样树突状细胞肿瘤(BPDCN)是一种侵袭性和罕见的血液系统恶性肿瘤,临床预后较差。干细胞移植有助于成人实现长期生存。然而,单倍同型干细胞移植(HID-SCT)与BPDCN化疗的益处尚不清楚。我们回顾性分析了32例诊断为BPDCN的患者,其中15例接受了HID-SCT, 17例仅接受了化疗。中位年龄52岁(范围19-78岁)。男女比例为2.2。皮肤、骨髓和淋巴结是最常见的三个受累部位。与化疗组相比,HID-SCT组患者的无进展生存期(PFS;中位数,7个月vs未达到,P
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来源期刊
Bone Marrow Transplantation
Bone Marrow Transplantation 医学-免疫学
CiteScore
8.40
自引率
8.30%
发文量
337
审稿时长
6 months
期刊介绍: Bone Marrow Transplantation publishes high quality, peer reviewed original research that addresses all aspects of basic biology and clinical use of haemopoietic stem cell transplantation. The broad scope of the journal thus encompasses topics such as stem cell biology, e.g., kinetics and cytokine control, transplantation immunology e.g., HLA and matching techniques, translational research, and clinical results of specific transplant protocols. Bone Marrow Transplantation publishes 24 issues a year.
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