Blastic plasmacytoid dendritic cell neoplasia

Blastic plasmacytoid dendritic cell neoplasia is a very rare, highly aggressive lymphatic systemic disease, which is derived from precursors of plasmacytoid dendritic cells . Cases of this lymphatic systemic disease have been reported after acute myeloid leukemia (AML).

Good initial response to combined chemotherapy. Experience is available with a combination of ifosphamide, methotrexate, etoposide, prednisolone and L-asparaginase. Recurrences are the rule; the average survival time is 12-14 months. A better prognosis can be expected with a solitary pattern of infestation. In this case, localized irradiation with fast electrons is recommended.

Since 2021 EU approval of the CD123 targeted therapy Tagraxofusp (Elzonris) for first-line treatment. Tagraxofusp is a fusion protein of a truncated diphtheria toxin and interleukin 3 (IL-3). The IL-3 domain binds to CD123 receptor molecules that are overexpressed by BPDCN cells and leads to the inhibition of protein biosynthesis and thus to apoptosis by releasing the diphtheria toxin inside the cell. The drug is used as monotherapy. The dosage form is intravenous.

1. Chang HJ et al (2012) A case of blastic plasmacytoid dendritic cell neoplasm initially mimicking cutaneous lupus erythematosus. Med Oncol 29:2417-2422
2. Cota C et al (2010) Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol 34: 75-87
3. Pileri A (2012) Blastic plasmacytoid dendritic cell neoplasm (BPDCN): the cutaneous sanctuary. G Ital Dermatol Venereol 147:603-608
4. Xie W et al (2012) Cutaneous blastic plasmacytoid dendritic cell neoplasm occurring after spontaneous remission of acute myeloid leukemia: a case report and review of literature. Med Oncol 29:2417-2422