title: Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric Population: A Case Series and Review of the Literature.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy primarily found in adults, often carrying a poor prognosis. There are only 33 reported pediatric cases of BPDCN in the literature. Although standard treatment is not yet established for children, current literature recommends the use of high-risk acute lymphoblastic leukemia (ALL)-type chemotherapy. Recent studies, however, have explored the benefits of combining chemotherapy with stem-cell transplantation. Here, the authors present 2 cases of pediatric BPDCN treated with different modalities. The first case is a 13-year-old girl who presented with a 3-month history of an initially asymptomatic firm nodule on her left shin. The second case is a 15-year-old boy who presented with a 4-month history of an enlarging subcutaneous nodule on the lower leg. Immunohistochemical staining of both patients was positive for markers consistent with BPDCN. The latter patient received ALL-type therapy alone, whereas the former received ALL-type chemotherapy and stem-cell transplantation. Since initial treatment, both patients remain disease-free. These cases contribute to the limited number of pediatric BPDCN cases, thus helping to advance our knowledge toward an optimal treatment protocol for clinical remission.
authors.affiliation: *Department of Dermatology, UC Irvine School of Medicine, Irvine, CA; †Department of Dermatology, Emory University School of Medicine, Atlanta, GA; ‡Department of Dermatology, UC Irvine Medical Center, Orange, CA; §Department of Dermatology, Kaiser Permanente, Los Angeles, CA; and ¶Department of Dermatology, Kaiser Permanente, Sunset Medical Center, Los Angeles, CA.
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Nguyen
authors.forename: Lauren
authors.initials: L
authors.lastname: Stuart
authors.forename: Hadas
authors.initials: H
authors.lastname: Skupsky
authors.forename: Yun-Sun
authors.initials: YS
authors.lastname: Lee
authors.forename: Arline
authors.initials: A
authors.lastname: Tsuchiya
authors.forename: David S
authors.initials: DS
authors.lastname: Cassarino
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D003713:Dendritic Cells / Q000473:pathology*; D005260:Female; D019337:Hematologic Neoplasms / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D016896:Treatment Outcome