Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

Özdemi̇r, Zehra; Seval, Güldane Cengiz; Şahin, Uğur; Uslu, Atilla; Gündüz, Mehmet; Bozdağ, Sinem Civriz; Toprak, Selami Koçak; Yüksel, Meltem Kurt; Topçuoğlu, Pervin; Kuzu, Işınsu; Özcan, Muhıt; Gürman, Günhan; İlhan, Osman

doi:10.1007/s12288-020-01313-9

Cited by 8 publications

(3 citation statements)

References 31 publications

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“…Given its good safety profile, venetoclax can be used in patients who are not eligible for tagraxofusp and HSCT. A few case reports have described the successful use of venetoclax in BPDCN patients [ 31 , 107 – 110 ]. Pemmaraju et al reported their experience with venetoclax and azacitidine in 10 patients (median age, 70 years) whose baseline co-morbidities, including renal failure, cardiac disease, and hypoalbuminemia, made them ineligible for tagraxofusp [ 111 ].…”

Section: Emerging Agentsmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Cuglievan

Connors

et al. 2023

Leukemia

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that can involve the bone marrow, peripheral blood, skin, lymph nodes, and the central nervous system. Though more common in older adults, BPDCN has been reported across all age groups, including infants and children. The incidence of pediatric BPDCN is extremely low and little is known about the disease. Pediatric BPDCN is believed to be clinically less aggressive but often with more dissemination at presentation than adult cases. Unlike adults who almost always proceed to a hematopoietic stem cell transplantation in first complete remission if transplant-eligible, the majority of children can be cured with a high-risk acute lymphoblastic leukemia-like regimen. Hematopoietic stem cell transplantation is recommended for children with high-risk disease, the definition of which continues to evolve, or those in relapse and refractory settings where outcomes continue to be dismal. Novel agents used in other hematologic malignancies and CD123 targeted agents, including chimeric antigen receptor T-cells and monoclonal/bispecific antibodies, are being brought into research and practice. Our goal is to provide a comprehensive review of presentation, diagnosis, and treatment by review of pediatric cases reported for the last 20 years, and a review of novel targeted therapies and therapies under investigation for adult and pediatric patients.

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Section: Emerging Agentsmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Cuglievan

Connors

et al. 2023

Leukemia

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“…Although BPDCN is classified in the context of AML, the onset of CNS involvement at diagnosis or during BPDCN is high 9,22,27,41,50–55 (Table 2).…”

Section: Recommendations and Proposalsmentioning

confidence: 99%

“…The incidence of CNS localization and its clinical impact is well known in pediatric and adult patients affected by ALL and in lymphomas, on the contrary, it is rarely reported in myeloproliferative disorders (ie, AML, MDS). 49 Although BPDCN is classified in the context of AML, the onset of CNS involvement at diagnosis or during BPDCN is high 9,22,27,41,[50][51][52][53][54][55] (Table 2).…”

Section: Ucn 7 Indication For Cns Prophylaxismentioning

confidence: 99%

Unmet Clinical Needs and Management Recommendations for Blastic Plasmacytoid Dendritic Cell Neoplasm: A Consensus-based Position Paper From an Ad Hoc International Expert Panel

et al. 2023

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with overall survival. The rarity of the disease results in a few large-scale studies, a lack of controlled clinical trials for its management, and a lack of evidence-based guidelines. Here, we present a review of unmet clinical needs on the management of BPDCN by a panel of eleven experts involved in the research and clinical practice of BPDCN. Recommendations and proposals were achieved by multiple-step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. The panel analyzed the critical issues of diagnostic pathway, prognostic stratification, therapy for young and fit patients and elderly and unfit patients, indication for allotransplant and for autotransplant, indication for central nervous system prophylaxis, and management of pediatric BPDCN patients. For each of these issues, consensus opinions were provided and, when appropriate, proposals for advancement in clinical practice were addressed. The hope is that this comprehensive overview will serve to improve the practice of BPDCN and inform the design and implementation of new studies in the field.

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Antineoplastics/methylprednisolone

2021

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Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

Cited by 8 publications

References 31 publications

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Unmet Clinical Needs and Management Recommendations for Blastic Plasmacytoid Dendritic Cell Neoplasm: A Consensus-based Position Paper From an Ad Hoc International Expert Panel

Antineoplastics/methylprednisolone

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