Universidad Peruana Cayetano Heredia

Blastic plasmacytoid dendritic cell neoplasm without cutaneous manifestation: A case report

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dc.contributor.author Razzeto, Alejandra
dc.contributor.author Garala, Pavan
dc.contributor.author Amoozgar, Behzad
dc.contributor.author Daliparty, Vasudev Malik
dc.contributor.author Rehman, Faseeha
dc.contributor.author Razzeto, Mariano
dc.date.accessioned 2022-02-01T21:18:27Z
dc.date.available 2022-02-01T21:18:27Z
dc.date.issued 2021
dc.identifier.uri https://hdl.handle.net/20.500.12866/11297
dc.description.abstract Background: As an uncommon malignancy with the highest prevalence in the elderly population, blastic plasmacytoid dendritic cell neoplasm or BPDCN is a hematologic disorder with unknown pathogenesis and devastating out-comes. This neoplasm usually manifests in the skin but can also involve the bone marrow, and less frequently the central nervous system (CNS). However, it does not exclude other organs and can even be associated with other malignancies. Here, we discuss an interesting case of BPDCN in an 85-year-old man who mainly presented with dizziness and weakness. Physical examination revealed splenomegaly, laboratory tests showed pancytopenia, and peripheral blood smear depicted metamyelocytes. Further workup including bone marrow biopsy revealed atypical cells and flow cytometry disclosed 84% blasts positive for cluster of differentiation (CD) 4, CD53, and CD156 suggestive of BPDCN. Moreover, cerebrospinal fluid (CSF) studies came back positive for tumor plasmacytoid dendritic cells. The patient underwent chemotherapy with CHOP, mini-CHOP regimens, and venetoclax, as well as treatment for CNS involvement. He achieved remission, but unfortunately had a recurrence of the disease. Later he was admitted due to pneumonia with concomitant recurrent pulmonary effusions complicated by multior-gan dysfunction and subsequently died. The diagnosis of BPDCN can be very challenging, and high clinical suspicion and intuition are required to reach the diagnosis, especially when patients do not present with cutaneous involvement. Concerning treatment op-tions, novel therapies such as tagraxofusp, a CD123-directed cytotoxin, are emerging in the hope of decreas-ing the rate of mortality for this aggressive malignancy en_US
dc.language.iso eng
dc.publisher International Scientific Literature
dc.relation.ispartofseries American Journal of Case Reports
dc.rights info:eu-repo/semantics/restrictedAccess
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es
dc.subject aged en_US
dc.subject Aged en_US
dc.subject human en_US
dc.subject Humans en_US
dc.subject male en_US
dc.subject Male en_US
dc.subject Aged, 80 and over en_US
dc.subject very elderly en_US
dc.subject complication en_US
dc.subject case report en_US
dc.subject skin en_US
dc.subject Skin en_US
dc.subject Neoplasm Recurrence, Local en_US
dc.subject tumor recurrence en_US
dc.subject Bone Marrow Neoplasms en_US
dc.subject dendritic cell en_US
dc.subject Dendritic Cells en_US
dc.subject hematologic disease en_US
dc.subject Hematologic Neoplasms en_US
dc.subject Leukemia en_US
dc.subject Rare Diseases en_US
dc.subject Skin Neoplasms en_US
dc.subject skin tumor en_US
dc.title Blastic plasmacytoid dendritic cell neoplasm without cutaneous manifestation: A case report en_US
dc.type info:eu-repo/semantics/article
dc.identifier.doi https://doi.org/10.12659/AJCR.932887
dc.relation.issn 1941-5923


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