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تسجيل

Blastic Plasmacytoid Dendritic Cell Neoplasm: Epidemiology

المؤلف:  Hoffman, R., Benz, E. J., Silberstein, L. E., Heslop, H., Weitz, J., & Salama, M. E.

المصدر:  Hematology : Basic Principles and Practice

الجزء والصفحة:  8th E , P1029

2026-11-04

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It remains challenging to accurately quantify the incidence of BPDCN due to the variety of names the disease had previously and because it was only recently given a code in the International Classification of Diseases (ICD). BPDCN incidence has been estimated by various sources as approximately 0.5% of all hematologic malignancies, 0.45/1,000,000 population, or 200 to 500 cases per year in the United States. The true incidence of BPDCN may be underestimated due to unfamiliarity with the disease among clinicians and pathologists, and because at least 20% of patients with BPDCN have a pre-existing myeloid neoplasm, such as chronic myelomonocytic leukemia (CMML), myelodysplastic syndrome (MDS), or AML. Careful clinical and pathological examination is necessary to distinguish between BPDCN and AML, particularly in patients with a known myeloid disorder (discussed in more detail later). In particular, BPDCN should be considered when patients with a myeloid neoplasm develop skin lesions. As recognition of BPDCN as a distinct entity improves and because there is an approved BPDCN-specific targeted therapy available and others in development, biopsy-confirmed incidence of the disease may continue to rise.

The median age of diagnosis in adult patients is approximately 60 to 65 years old. The disease also occurs in children and there is a bimodal incidence pattern, similar to what is observed in B-cell acute lymphoblastic leukemia, with a separate pediatric peak of BPDCN incidence at age 8 to 10 years. There is a striking male predominance in adults with BPDCN, with men affected 3 to 4 times more frequently than women. The disease is not nearly as gender-biased in children, where the male incidence is only 1.2 times higher than in females. There is no exposure history clearly linked to BPDCN, such as prior chemotherapy, radiation, or environmental toxins. Different from conventional dendritic cells (cDCs), which present processed antigens to lymphocytes, pDCs’ primary role is to sense pathogens and activate the immune system by producing interferon α and β. Thus, there has been interest in determining if BPDCN has an infectious etiology. However, there has been no infection linked to BPDCN, neither in empirical assessment of specific viruses, such as Epstein-Barr virus, nor in unbiased microbial metagenomics using next-generation sequencing.