Chemotherapy Options for Blastic Plasmacytoid Dendritic Cell Neoplasm

Key points

Intensive chemotherapy for fit individuals

Given the lack of prospective trials of induction regimens for BPDCN, the best recommendation for treatments used for induction are based on retrospective analyses of patients treated in routine clinical practice. In our experience, whether the patient was referred to a certain subspecialist influenced the treatment decision.⁴ For example, if referred to a lymphoma specialist the patients tended to get lymphoma regimens, for example, CHOP; however, if referred to a leukemia specialist the

Non-fit individuals

For patients with BDCN who cannot tolerate aggressive therapy, there is limited data for lower-intensity chemotherapy options. As discussed above, Reimer and colleagues⁵ found that those receiving therapies considered less intensive than CHOP had very poor outcomes (median overall survival = 9 months) with only 7% reporting sustained remissions and no patients with long-term survival. The median age in this group was 79 suggesting that they were likely not eligible for hematopoietic stem cell

Summary

Although BPDCN often presents with skin findings initially, it can be mistaken for non-neoplastic causes resulting in delayed diagnosis. Unless the correct pathologic workup is performed it could also be misdiagnosed for other neoplastic entities resulting in incorrect or delayed treatment. This highlights the need for increased awareness of the disease and the importance of having reliable immunohistochemical and flow cytometry markers, discussed elsewhere in this review series. If BPDCN is

Disclosure

The authors have nothing to disclose.