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Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy derived from type 2 Dendritic cells (immature Plasmacytoid dendritic cells). It is an aggressive lymphoma and most commonly presents as nonpruritic cutaneous lesions. It can also involve the bone marrow, lymph node...

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Autores principales: Al-Alwan, Ahmad, Khalid, Farhan, Vyas, Charmee, Sirpal, Vishakha, Bader, Husam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Greater Baltimore Medical Center 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10589008/
https://www.ncbi.nlm.nih.gov/pubmed/37868236
http://dx.doi.org/10.55729/2000-9666.1208
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author Al-Alwan, Ahmad
Khalid, Farhan
Vyas, Charmee
Sirpal, Vishakha
Bader, Husam
author_facet Al-Alwan, Ahmad
Khalid, Farhan
Vyas, Charmee
Sirpal, Vishakha
Bader, Husam
author_sort Al-Alwan, Ahmad
collection PubMed
description Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy derived from type 2 Dendritic cells (immature Plasmacytoid dendritic cells). It is an aggressive lymphoma and most commonly presents as nonpruritic cutaneous lesions. It can also involve the bone marrow, lymph nodes, or circulating peripheral blasts. Here we present a 61-year-old female with skin bruises all over her body for the last three months associated with fatigue, night sweats, and unintentional weight loss. Her initial diagnosis was Acute Myeloid Leukemia (AML), but later she was diagnosed with BPDCN on tumor biopsy consistent with CD56+ neoplasm. The patient was treated with cyclophosphamide with steroid bridge. She was follow-up every week for the disseminated intravascular coagulation panel and monitored for tumor lysis syndrome. The management of the BPDCN is still unclear due to the condition’s rarity. tagraxofusp has been used for remission induction as it has a higher response rate with an acceptable toxicity profile than conventional chemotherapy. Allogeneic hematopoietic stem cell transplantation (HCT) is recommended in patients with the first remission. For patients with relapsed/refractory disease, tagraxofusp demonstrates a good overall response, followed by HCT.
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spelling pubmed-105890082023-10-21 Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case Al-Alwan, Ahmad Khalid, Farhan Vyas, Charmee Sirpal, Vishakha Bader, Husam J Community Hosp Intern Med Perspect Case Report Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy derived from type 2 Dendritic cells (immature Plasmacytoid dendritic cells). It is an aggressive lymphoma and most commonly presents as nonpruritic cutaneous lesions. It can also involve the bone marrow, lymph nodes, or circulating peripheral blasts. Here we present a 61-year-old female with skin bruises all over her body for the last three months associated with fatigue, night sweats, and unintentional weight loss. Her initial diagnosis was Acute Myeloid Leukemia (AML), but later she was diagnosed with BPDCN on tumor biopsy consistent with CD56+ neoplasm. The patient was treated with cyclophosphamide with steroid bridge. She was follow-up every week for the disseminated intravascular coagulation panel and monitored for tumor lysis syndrome. The management of the BPDCN is still unclear due to the condition’s rarity. tagraxofusp has been used for remission induction as it has a higher response rate with an acceptable toxicity profile than conventional chemotherapy. Allogeneic hematopoietic stem cell transplantation (HCT) is recommended in patients with the first remission. For patients with relapsed/refractory disease, tagraxofusp demonstrates a good overall response, followed by HCT. Greater Baltimore Medical Center 2023-06-29 /pmc/articles/PMC10589008/ /pubmed/37868236 http://dx.doi.org/10.55729/2000-9666.1208 Text en © 2023 Greater Baltimore Medical Center https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the CC BY-NC license (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ).
spellingShingle Case Report
Al-Alwan, Ahmad
Khalid, Farhan
Vyas, Charmee
Sirpal, Vishakha
Bader, Husam
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title_full Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title_fullStr Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title_full_unstemmed Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title_short Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) in an Elderly Female: A Rare Case
title_sort blastic plasmacytoid dendritic cell neoplasm (bpdcn) in an elderly female: a rare case
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10589008/
https://www.ncbi.nlm.nih.gov/pubmed/37868236
http://dx.doi.org/10.55729/2000-9666.1208
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