Blastic plasmacytoid dendritic cell neoplasm with cutaneous presentation: A case series in children

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy that can present with multiorgan involvement including the skin. While this entity accounts for 0.1% of all cutaneous lymphomas in adults, fewer than 90 pediatric cases have been described in the literature. We present three cases of pediatric BPDCN, highlighting the variable cutaneous and clinical presentation.

A rapidly growing nodule on the leg

“…BPDCN is a rare and clinically aggressive hematologic malignancy derived from pDC precursors, which normally accumulate in lymph nodes in response to antigens from viral infections or autoimmune diseases 1 . Although it most commonly presents in males older than 65 years, it has been very rarely described in pediatric populations 2 . Approximately 50%–66% of patients with BPDCN exhibit genetic abnormalities, including loss of tumor suppressor genes and mutations characteristic of myelodysplastic syndrome 3 .…”

“…1 Although it most commonly presents in males older than 65 years, it has been very rarely described in pediatric populations. 2 Approximately 50%-66% of patients with BPDCN exhibit genetic abnormalities, including loss of tumor suppressor genes and mutations characteristic of myelodysplastic syndrome. 3 Developmental ontogeny studies suggest that the initial malignant transformation seen in pDCs may start in the skin prior to dissemination to the bone marrow.…”