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Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases

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...

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Autores principales: Guo, Jiang-Hong, Zhang, Hong-Wei, Wang, Li, Bai, Wei, Wang, Jin-Fen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8638054/
https://www.ncbi.nlm.nih.gov/pubmed/34904102
http://dx.doi.org/10.12998/wjcc.v9.i33.10293
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author Guo, Jiang-Hong
Zhang, Hong-Wei
Wang, Li
Bai, Wei
Wang, Jin-Fen
author_facet Guo, Jiang-Hong
Zhang, Hong-Wei
Wang, Li
Bai, Wei
Wang, Jin-Fen
author_sort Guo, Jiang-Hong
collection PubMed
description 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.
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spelling pubmed-86380542021-12-12 Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases Guo, Jiang-Hong Zhang, Hong-Wei Wang, Li Bai, Wei Wang, Jin-Fen World J Clin Cases Case Report 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. Baishideng Publishing Group Inc 2021-11-26 2021-11-26 /pmc/articles/PMC8638054/ /pubmed/34904102 http://dx.doi.org/10.12998/wjcc.v9.i33.10293 Text en ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved. https://creativecommons.org/licenses/by-nc/4.0/This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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.
spellingShingle Case Report
Guo, Jiang-Hong
Zhang, Hong-Wei
Wang, Li
Bai, Wei
Wang, Jin-Fen
Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title_full Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title_fullStr Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title_full_unstemmed Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title_short Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases
title_sort blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: report of three cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8638054/
https://www.ncbi.nlm.nih.gov/pubmed/34904102
http://dx.doi.org/10.12998/wjcc.v9.i33.10293
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