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Review
. 2025 Jan 11;17(2):221.
doi: 10.3390/cancers17020221.

A Multidisciplinary Approach to Diagnosing Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Practical Recommendations and Insights from Countries of the Gulf Cooperation Council

Nasir Bakshi  1 , Ayman Al Hejazi  2 , Hatim Al-Maghraby  3 , Areej Al Mugairi  4 , Ahmad S Alotaibi  5 , Haitham Khogeer  1 , Rania Medhat Seliem  6 , Ramesh Pandita  7 , Heba Raslan  8 , Phyu P Aung  9 , Robert S Ohgami  10
Affiliations
Review

A Multidisciplinary Approach to Diagnosing Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Practical Recommendations and Insights from Countries of the Gulf Cooperation Council

Nasir Bakshi et al. Cancers (Basel). .

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive orphan hematopoietic malignancy characterized by cutaneous and systemic hematologic involvement. BPDCN is frequently misidentified, but early, accurate diagnosis is critical to extending patient survival using tagraxofusp, a first-in-class CD123-targeted therapy, and increasing their chances of receiving a potentially curative stem cell transplantation. Cases of BPDCN in countries of the Gulf Cooperation Council are lower than the extrapolated incidence from other geographic locations due to lack of awareness of key diagnostic features, lack of consensus on the minimal phenotype for diagnosis, and lack of local immunohistochemistry testing facilities, contributing to underdiagnosis in this region. Practical recommendations, a streamlined diagnostic panel, and suggested multidisciplinary approaches based on expert experience regarding diagnostic and clinical challenges specific to this region, and a review of the literature are presented here to facilitate diagnosis of BPDCN in this region by primary care physicians, dermatologists, and hematologists.

Keywords: Gulf Cooperation Council; blastic plasmacytoid dendritic cell neoplasm; consensus recommendations; dendritic cells/pathology; differential diagnosis; hematologic neoplasms/diagnosis; tagraxofusp.

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Conflict of interest statement

N.B. has received consulting fees from Stemline Therapeutics and travel expenses from BD Biosciences. R.P. has received honoraria from Janssen, Amgen, Novartis, Sanofi, AstraZeneca, and Takeda. She has also received consulting fees from Janssen, Servier, Amgen, Sobi, Novartis, and Pfizer and speakers’ bureau fees from Amgen, Biologics, AstraZeneca, New Bridge, and Pfizer. P.P.A. has received research funding from the Melanoma Research Alliance. R.S.O. has received honoraria and speakers’ bureau payments from Recordati. He has also received travel expenses from Beckman Coulter and Recordati. A.A.H., H.A-M., A.A.M., H.R., and R.M.S. have no conflicts to report.

Figures

Figure 2
Figure 2
Proposed BPDCN diagnostic pathway for patients referred with skin lesions only. Required and strongly recommended options are shown with the solid arrows, while dashed arrows show ancillary studies that are not always required but may be useful.
Figure 3
Figure 3
Proposed BPDCN diagnostic pathway for patients referred with bone marrow and blood involvement only. Required and strongly recommended options are shown with the solid arrows, while dashed arrows show ancillary studies that are not always required but may be useful.
Figure 4
Figure 4
Proposed BPDCN diagnostic pathway for patients referred with skin lesions, bone marrow involvement, and lymphadenopathy. Required and strongly recommended options are shown with the solid arrows, while dashed arrows show ancillary studies that are not always required but may be useful.
Figure 1
Figure 1
Map of the Countries in the Gulf Cooperation Council. National population figures as of 2021 [23] and the number of cases diagnosed in past 5 years are shown. • Location of represented hospitals. * n/a = not applicable.
See this image and copyright information in PMC

References

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