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Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report

BACKGROUND: The present case contributes to the limited literature on central nervous system involvement of blastic plasmacytoid dendritic cell neoplasm (BPDCN).  CASE PRESENTATION : A 63-year-old male presented to the department of neurology with a three-day history of rapidly progressing headache,...

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Autores principales: Florescu, Anna Maria, Sørensen, Anne Louise Tølbøll, Nielsen, Henrik Vedel, Tolnai, Daniel, Sjö, Lene Dissing, Larsen, Katja Lohmann, Al-Karagholi, Mohammad Al-Mahdi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9229753/
https://www.ncbi.nlm.nih.gov/pubmed/35751052
http://dx.doi.org/10.1186/s12883-022-02748-5
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author Florescu, Anna Maria
Sørensen, Anne Louise Tølbøll
Nielsen, Henrik Vedel
Tolnai, Daniel
Sjö, Lene Dissing
Larsen, Katja Lohmann
Al-Karagholi, Mohammad Al-Mahdi
author_facet Florescu, Anna Maria
Sørensen, Anne Louise Tølbøll
Nielsen, Henrik Vedel
Tolnai, Daniel
Sjö, Lene Dissing
Larsen, Katja Lohmann
Al-Karagholi, Mohammad Al-Mahdi
author_sort Florescu, Anna Maria
collection PubMed
description BACKGROUND: The present case contributes to the limited literature on central nervous system involvement of blastic plasmacytoid dendritic cell neoplasm (BPDCN).  CASE PRESENTATION : A 63-year-old male presented to the department of neurology with a three-day history of rapidly progressing headache, fatigue, and confusion. Physical examination revealed multiple bruise-like skin lesions. Initial laboratory workup raised suspicion of acute leukemia, and a brain computer tomography identified several hyperdense processes. A bone marrow biopsy gave the diagnosis BPDCN, a rare and aggressive hematologic malignancy derived from plasmacytoid dendritic cells with a poor prognosis. Lumbar puncture showed not only signs of BPDCN, but also cerebral toxoplasmosis, thus providing a differential diagnosis. Despite intensive systemic and intrathecal chemotherapy, the patient died 25 days later due to multi-organ failure. DISCUSSION: The exact incidence of BPDCN is unknown and perhaps underestimated but may account for 0.5 – 1% of all hematological malignancies. The median age at onset is 60 to 70 years, and most patients are men. Cutaneous lesions are the most frequent clinical manifestation at diagnosis. Other symptoms present at time of diagnosis or during disease progression include lymphadenopathy, splenomegaly and cytopenia caused by bone marrow involvement. Although the majority of BPDCN patients have no symptoms or signs of central nervous system involvement, plasmacytoid dendritic cells have been detected in the cerebrospinal fluid in more than 50%. CONCLUSIONS: This case highlights the importance of considering hematological malignancies as a differential diagnosis in patients developing acute neurological symptoms and raises suspicion of a possible association between toxoplasmosis and hematological malignancies.
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spelling pubmed-92297532022-06-25 Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report Florescu, Anna Maria Sørensen, Anne Louise Tølbøll Nielsen, Henrik Vedel Tolnai, Daniel Sjö, Lene Dissing Larsen, Katja Lohmann Al-Karagholi, Mohammad Al-Mahdi BMC Neurol Case Report BACKGROUND: The present case contributes to the limited literature on central nervous system involvement of blastic plasmacytoid dendritic cell neoplasm (BPDCN).  CASE PRESENTATION : A 63-year-old male presented to the department of neurology with a three-day history of rapidly progressing headache, fatigue, and confusion. Physical examination revealed multiple bruise-like skin lesions. Initial laboratory workup raised suspicion of acute leukemia, and a brain computer tomography identified several hyperdense processes. A bone marrow biopsy gave the diagnosis BPDCN, a rare and aggressive hematologic malignancy derived from plasmacytoid dendritic cells with a poor prognosis. Lumbar puncture showed not only signs of BPDCN, but also cerebral toxoplasmosis, thus providing a differential diagnosis. Despite intensive systemic and intrathecal chemotherapy, the patient died 25 days later due to multi-organ failure. DISCUSSION: The exact incidence of BPDCN is unknown and perhaps underestimated but may account for 0.5 – 1% of all hematological malignancies. The median age at onset is 60 to 70 years, and most patients are men. Cutaneous lesions are the most frequent clinical manifestation at diagnosis. Other symptoms present at time of diagnosis or during disease progression include lymphadenopathy, splenomegaly and cytopenia caused by bone marrow involvement. Although the majority of BPDCN patients have no symptoms or signs of central nervous system involvement, plasmacytoid dendritic cells have been detected in the cerebrospinal fluid in more than 50%. CONCLUSIONS: This case highlights the importance of considering hematological malignancies as a differential diagnosis in patients developing acute neurological symptoms and raises suspicion of a possible association between toxoplasmosis and hematological malignancies. BioMed Central 2022-06-24 /pmc/articles/PMC9229753/ /pubmed/35751052 http://dx.doi.org/10.1186/s12883-022-02748-5 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Florescu, Anna Maria
Sørensen, Anne Louise Tølbøll
Nielsen, Henrik Vedel
Tolnai, Daniel
Sjö, Lene Dissing
Larsen, Katja Lohmann
Al-Karagholi, Mohammad Al-Mahdi
Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title_full Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title_fullStr Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title_full_unstemmed Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title_short Blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
title_sort blastic plasmacytoid dendritic cell neoplasm and cerebral toxoplasmosis: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9229753/
https://www.ncbi.nlm.nih.gov/pubmed/35751052
http://dx.doi.org/10.1186/s12883-022-02748-5
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