Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 6, 2024; 12(1): 136-141
Published online Jan 6, 2024. doi: 10.12998/wjcc.v12.i1.136
Azacitidine maintenance therapy for blastic plasmacytoid dendritic cell neoplasm allograft: A case report
Li-Li Tao, Hui-Ting Wen, Zi-Yi Wang, Juan Cheng, Li Zhao
Li-Li Tao, Zi-Yi Wang, Department of Hematology, The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
Hui-Ting Wen, Juan Cheng, Li Zhao, Department of Hematology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
Author contributions: Tao LL designed the study and wrote the manuscript; Wen HT and Wang ZY collected and analyzed the data; Tao LL and Cheng J prepared figures; Cheng J and Zhao L was in charge of patient treatment and designed the paper; All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Li Zhao, Doctor, MD, Chief Doctor, Department of Hematology, The First Affiliated Hospital, Lanzhou University, No. 1 Donggangxi Road, Chengguan District, Lanzhou 730000, Gansu Province, China. zhaoli@lzu.edu.cn
Received: August 25, 2023
Peer-review started: August 25, 2023
First decision: December 7, 2023
Revised: December 12, 2023
Accepted: December 19, 2023
Article in press: December 19, 2023
Published online: January 6, 2024
Processing time: 130 Days and 3.7 Hours
Abstract
BACKGROUND

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly invasive malignant neoplasm. There is no universally accepted standard of care because of its rarity and the dearth of prospective research. It is still challenging for some patients to achieve persistent clinical remission or cure, despite the success of allogeneic hematopoietic stem cell transplantation (allo-HSCT), indicating that there is still a significant recurrence rate. We report a case of prevention of BPDCN allograft recurrence by azacitidine maintenance therapy and review the relevant literature.

CASE SUMMARY

We report a 41-year-old man with BPDCN who was admitted to hospital due to skin sclerosis for > 5 mo’ duration. BPDCN was diagnosed by combined clinical assessment and laboratory examinations. Following diagnosis, the patients underwent induction consolidation chemotherapy to achieve the first complete remission, followed by bridging allo-HSCT. Post-transplantation, azacitidine (75 mg/m2 for 7 d) was administered as maintenance therapy, with repeat administration every 4–6 wk and appropriate extension of the chemotherapy cycle. After 10 cycles, the patient has been disease free for 26 mo after transplantation. Regular assessments of bone marrow morphology, minimal residual disease, full donor chimerism, Epstein–Barr virus, and cytomegalovirus all yielded normal results with no abnormalities detected.

CONCLUSION

Azacitidine may be a safe and effective maintenance treatment for BPDCN following transplantation because there were no overt adverse events during the course of treatment.

Keywords: Blastic plasmacytoid dendritic cell neoplasm, Azacitidine, Allogeneic hematopoietic stem cell transplantation, Maintenance therapy, Case report

Core Tip: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological tumor with cutaneous invasion as the first clinical manifestation, as well as lymph nodes, soft tissues, peripheral blood, and bone marrow involvement. The first recommended treatment option is allogeneic hematopoietic stem cell transplantation, but there is still a high recurrence rate after transplantation. We present a case of azacitidine maintenance treatment to prevent BPDCN allograft relapse in our center.