Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: A subset with possible durable remission without transplantation

ALL: acute lymphoblastic leukemia BPDCN: blastic plasmacytoid dendritic cell neoplasm CVAD: cyclophosphamide, vincristine, doxorubicin, dexamethasone CVAD A1B: cyclophosphamide, vincristine, doxorubicin, dexamethasone alternating with methotrexate and cytarabine HSCT: hematopoietic stem cell transplantation LRT: localized radiotherapy LS-BPDCN: localized skin-limited blastic plasmacytoid dendritic cell neoplasm INTRODUCTION Blastic plasmacytoid dendritic cell neoplasm (BPDCN) was defined in the 2008 World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissue as a malignant proliferation of blastic plasmacytoid dendritic cells. The skin lesions appear as bruiselike erythematous nodules or tumors and are usually found already at presentation. BPDCN is highly malignant, with a mean survival time of 15.2 months. Data are limited concerning patients with localized skin-limited BPDCN (LS-BPDCN). We report our experience with 2 patients with LSBPDCN, both of whom achieved potential cure with hyper-CVAD chemotherapy (cyclophosphamide, vincristine, doxorubicin, dexamethasone) followed by consolidative localized radiotherapy (LRT), without hematopoietic stem cell transplantation (HSCT).