Effective Treatment of a Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm with a Cutaneous Tumor Alone by Stem Cell Transplantation with Reduced Intensity Conditioning

Abstract

Pediatric blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy that has an extremely poor prognosis despite the use of intensive chemotherapy. Recently, treatment of BPDCN with bone marrow transplantation (BMT) using myeloablative conditioning has been reported to increase survival in adults. We report a 9-year-old girl with cutaneous BPDCN who was successfully treated with combination chemotherapy followed by BMT using reduced intensity conditioning (RIC), without any adverse complications. The success of this treatment regimen suggests that BMT with RIC may be a feasible option for treating children with cutaneous BPDCN.

A new palliative treatment for blastic plasmacytoid dendritic cell neoplasm: a case report and review of the literature

“…21 Many other new therapy modalities are also being investigated. For instance, stimulation of liver X receptors (LXRs) has been shown to reduce BPDCN spleen and bone marrow infiltrations; 22 reduced intensity conditioning (RIC) regimens have been used to treat elderly and paediatric patients with BPDCN to decrease treatment-related toxicities; 23 early detection strategies may be applied in patients with a previous history of haematologic malignancy in order to provide early treatment before it transforms into BPDCN; 24 and venetoclax, an orally bioavailable inhibitor of BCL-2, has been used to treat patients with BPDCN. 25 In the present case, a new palliative treatment that comprised a combination of gemcitabine, nedaplatin and bleomycin was administered to an elderly patient with BPDCN who had a history of other diseases.…”

Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm

“…Rather than hematopoietic SCT after first complete remission as in adults, SCT is usually reserved for children who relapse and achieve a second remission . Nevertheless, given the rarity of this condition, there is a paucity of evidence‐based data to guide treatment, and described approaches are limited to small studies …”