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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2020; 41(05): 761-763
DOI: 10.4103/ijmpo.ijmpo_143_19
Practitioner Section

Blastic Plasmacytoid Dendritic Cell Neoplasm in a Young Female: Rare and Challenging

Ketan Modak
1   Department of Clinical Haematology and Bone Marrow Transplantation, Tata Medical Center, Kolkata, West Bengal, India
,
Jeevan Garg
1   Department of Clinical Haematology and Bone Marrow Transplantation, Tata Medical Center, Kolkata, West Bengal, India
,
Deepak Mishra
2   Department of Laboratory Haematology, Tata Medical Center, Kolkata, West Bengal, India
,
Vivek Radhakrishnan
1   Department of Clinical Haematology and Bone Marrow Transplantation, Tata Medical Center, Kolkata, West Bengal, India
› Author Affiliations Financial support and sponsorship Nil.
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Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic malignancy with an aggressive behavior, seen commonly in the elderly age group and usually involves the skin. Evaluation, diagnosis, and treatment pose unique challenges with poor outcomes. We report a case of an 18-year-old female who presented with lymphadenopathy and bicytopenia. The diagnosis of BPDCN was an initial challenge. She thereafter received intensive chemotherapy followed by an allogenic partially mismatched sibling donor hematopoietic cell transplantation, only to relapse in a few months. Beginning from the diagnosis, the management of this rare disorder and its relapse until her last followup provided several learning opportunities.

Keywords

Blastic plasmacytoid dendritic cell neoplasm - CD123 - hematopoietic cell transplant


Publication History

Received: 17 June 2019

Accepted: 14 October 2019

Article published online:
17 May 2021

© 2020. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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