Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric Population

Abstract

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.

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

“…Blastic plasmacytoid dendritic cell neoplasm typically presents with cutaneous findings at diagnosis ranging from single or multiple nontender, sometimes pruritic, erythematous to violaceous papules, plaques, or nodules . Approximately 85% of adult cases and 76% of pediatric cases present with skin findings .…”

“…It is exceptionally rare in children, with fewer than 75 reported cases in the literature . Clinical manifestations are variable and include single or multiple, nontender, sometimes pruritic, erythematous to violaceous papules, plaques, or nodules . We present a rare case of pediatric BPDCN without cutaneous findings at the time of diagnosis, although they appeared after initiation of treatment.…”

“…Blastic plasmacytoid dendritic cell neoplasm (BPDCN), also known as CD4 + /CD56 + hematodermic neoplasm or blastic natural killer cell lymphoma, is a rare neoplasm, accounting for less than 1% of cutaneous lymphomas . It is thought to arise from precursors of plasmacytoid dendritic cells . This malignancy typically affects older men (median age 67 years) .…”

“…It is thought to arise from precursors of plasmacytoid dendritic cells . This malignancy typically affects older men (median age 67 years) . It is exceptionally rare in children, with fewer than 75 reported cases in the literature .…”

“…This malignancy typically affects older men (median age 67 years) . It is exceptionally rare in children, with fewer than 75 reported cases in the literature . Clinical manifestations are variable and include single or multiple, nontender, sometimes pruritic, erythematous to violaceous papules, plaques, or nodules .…”