Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2021; 9(33): 10293-10299
Published online Nov 26, 2021. doi: 10.12998/wjcc.v9.i33.10293
Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
Jiang-Hong Guo, Hong-Wei Zhang, Li Wang, Wei Bai, Jin-Fen Wang
Jiang-Hong Guo, Li Wang, Wei Bai, Jin-Fen Wang, Department of Pathology, Shanxi Cancer Hospital, Taiyuan 030000, Shanxi Province, China
Hong-Wei Zhang, Department of Hematology, Shanxi Cancer Hospital, Taiyuan 030000, Shanxi Province, China
Author contributions: Guo JH reviewed the literature; Zhang HW was the patient’s physician; Guo JH and Zhang HW drafted the manuscript; Wang JF contributed to data collection; Wang L and Bai W were responsible for revising the manuscript for important content; and all authors issued final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patients for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016). The manuscript was prepared and revised according to the 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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jin-Fen Wang, MS, Chief Doctor, Department of Pathology, Shanxi Cancer Hospital, No. 3 Kaixuan Street, Xinghualing District, Taiyuan 030000, Shanxi Province, China. jinfenwang2021@163.com
Received: July 7, 2021
Peer-review started: July 7, 2021
First decision: July 15, 2021
Revised: July 27, 2021
Accepted: August 30, 2021
Article in press: August 30, 2021
Published online: November 26, 2021
Processing time: 138 Days and 0.3 Hours
Abstract
BACKGROUND

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematopoietic malignancy. BPDCN is difficult to diagnose because of the overlap in morphologic and immunophenotypic features with various cutaneous lymphatic hematopoietic tumors.

CASE SUMMARY

We report on three BPDCN cases, all characterized by skin nodules and examined by histology, immunohistochemical detection, in situ hybridization for Epstein-Barr virus, and follow-up. We also review the relevant literature. All patients were positive for CD56 and negative for Epstein-Barr encoded small RNA. Two patients had bone marrow involvement. Chemotherapy is the main treatment for BPDCN, but case 1 showed bone marrow suppression and case 2 developed recurrence after chemotherapy. Case 1 survived for 7 mo, case 2 for 17 mo, and case 3 for 9 mo.

CONCLUSION

An accurate pathological diagnosis is a precondition for treatment, and the diagnosis of BPDCN should be based on a combination of clinical symptoms, pathological characteristics, immunophenotype, and other auxiliary examinations. It is necessary to clarify the clinicopathological features and biological behavior of BPDCN to improve its understanding by both clinicians and pathologists. Case 2 survived significantly longer than the other two cases, suggesting that the treatment received by case 2 was more effective.

Keywords: Blastic plasmacytoid dendritic cell neoplasm; Diagnosis; Immunohistochemistry; Skin lesion; Follow-up; Case report

Core Tip: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is difficult to diagnose because of the overlap in morphologic and immunophenotypic features with various cutaneous lymphatic hematopoietic tumors. We report the clinical symptoms, pathological characteristics, immunophenotype, treatment, and follow-up (from diagnosis until death) for three patients with BPDCN. It is necessary to clarify the clinicopathological features and biological behavior of BPDCN to improve the understanding of the disease by both clinicians and pathologists. The survival time of case 2 was significantly longer than usual, suggesting that the treatment received by this case was suitable for clinical application.