Blastic plasmacytoid dendritic cell neoplasia

Synonym(s)

blastic plasmacytoid dendritic cell neoplasm; BPDCN

Definition
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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).

Clinical features
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Clinically dermatologically, patients usually present with exanthematic, reddish-brown, asymptomatic plaques and nodules. Clinically similarities to systemic lupus erythematosus can be found. Less frequently solitary nodules or plaques are found. Systemic lupus erythematosus is regularly found with infestation of bone marrow, lymph nodes and peripheral blood. A purely cutaneous form is rarer.

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Histology
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Dense perivascular or periadnexial infiltrate of blasts with irregular hyperchromatic sometimes twisted nuclei, with markers for CD4, CD43, CD45, CD56, CD68 (regular detection) and CD123. No evidence of Epstein-Barr Virus (EBV).

Therapy
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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.

Note(s)
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In blast plasmacytoid dendritic cell neoplasia, genetic variations are often found, especially losses of those genes that have a control function in the cell cycle. Often aberrations are found in the p27 gene, which is located on chromosome 12.

Literature
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Chang HJ et al (2012) A case of blastic plasmacytoid dendritic cell neoplasm initially mimicking cutaneous lupus erythematosus. Med Oncol 29:2417-2422
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
Pileri A (2012) Blastic plasmacytoid dendritic cell neoplasm (BPDCN): the cutaneous sanctuary. G Ital Dermatol Venereol 147:603-608
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