Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were children, and 283 were adults aged 19 or over. Age was shown to be an independent prognostic factor predictive of more favorable outcomes across measures including initial response to therapy, likelihood of relapse, and overall survival at follow-up. The distribution of affected organs at diagnosis was similar across children and adults and type of clinical presentation did not disproportionately influence 1 age group's prognosis over the other. Acute lymphoblastic leukemia-type chemotherapy regimens were shown to be superior to other chemotherapy regimens (acute myeloid leukemia, lymphoma, acute lymphoblastic leukemia/lymphoma, other, or none) in inducing complete remission. Allogeneic stem cell transplantation was shown to increase mean survival time. Future research may be directed toward elucidating the further morphologic, cytogenetic, and cytochemical differences between younger and older BPDCN patients.

Putative new childhood leukemia cancer predisposition syndrome caused by germline bi‐allelic missense mutations in DDX41

“…In 2008, BPDCN was reclassified by WHO as an AML‐related precursor neoplasm, and can be classified by its pattern of cell surface markers . In a recent review of 357 cases, 74 cases were below age 18 years . In the pediatric cohort sex distribution was nearly equal (females 45%), whereas only 26% were females in the adult cohort.…”

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

“…A recent literature review evaluating adult's children's responses to first‐line therapy found that older age and hematologic organ involvement at presentation were associated with worse outcomes, acute lymphoblastic leukemia–type chemotherapy was tied to better outcomes, and sex was not predictive of treatment response. Although the presence of cutaneous lesions at diagnosis was not significantly associated with response to first‐line therapy, it was correlated with worse prognosis at 18‐month follow‐up after initial remission …”

“…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 .…”

Exploring the effect of chidamide on blastic plasmacytoid dendritic cell neoplasm: a case report and literature review

“…It is highly responsive to chemotherapy used for ALL, acute myeloid leukemia (AML) and non-Hodgkin lymphoma, but the median event-free survival is only 10 months. 4 Some patients received long-term survival after allo-SCT, especially children, but there were still 30%–40% of them who experienced relapse even after performing allo-SCT. 5 Hence, the understanding of the pathobiology of BPDCN and the development of novel agents is urgently demanded.…”