Dendritic Cell Leukemia: a Review
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LEUKEMIA (A AGUAYO, SECTION EDITOR)
Dendritic Cell Leukemia: a Review Nikolaos J. Tsagarakis 1 & Georgios Paterakis 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review The purpose of this review was to summarize the clinical, diagnostic, and therapeutic features of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Recent Findings Several case reports and series revealed new clinical, molecular, diagnostic, and therapeutic aspects of the disease. The clinical presentation diversity has been confirmed, with frequent leukemic non-cutaneous or rare atypical manifestations. The clonal evolution in the development of BPDCN has not been sufficiently elucidated. Although certain immunophenotypic markers (CD4, TCL1, CD123, CD56, CD303) are indicative of BPDCN, the diagnosis remains in certain cases challenging. Adult (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (HSCT) is related to a favorable outcome, while chemotherapy alone seems enough in children. Future studies should continue to investigate whether CD123-directed therapies could be utilized. Summary BPDCN is a rare aggressive malignancy that needs an aggressive induction therapy. Although a diagnostic consensus is still lacking, and large retrospective studies are also needed to obtain standardized treatment guidelines, the future perspectives are encouraging, because of novel therapeutic agents that are under investigation. Keywords Blastic plasmacytoid dendritic cell neoplasm . BPDCN review . Dendritic cell leukemia . BPDCN diagnosis . Immunophenotype . Therapy
Introduction Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is recognized in the WHO 2016 classification of hematopoietic neoplasms as a distinct acute leukemia entity [1]. Described for the first time in 1994 as CD4+ cutaneous lymphoma with high expression of CD56, BPDCN has been known previously with various names such as blastic natural killer (NK) leukemia/lymphoma, agranular CD4+/CD56+ hematodermic neoplasm, and agranular CD4+ NK cell leukemia [2]. It is a rare disease presenting across all ages with either skin or both skin and bone marrow involvement often conferring a poor prognosis [3]. BPDCN is often associated with a complex karyotype, frequent deletions of tumor suppressor genes, and
This article is part of the Topical Collection on Leukemia * Nikolaos J. Tsagarakis [email protected] 1
Department of Immunology, “G. Gennimatas” General Hospital, Mesogion Avenue 154, 11527 Athens, Greece
mutations affecting either the DNA methylation or chromatin remodeling pathways [4].
Origin of the Neoplastic Cell The potential of the transformed (leukemic) multipotential hematopoietic cell to differentiate and mature along any myeloid lineage forms the basis for the phenotypic classification of acute and chronic myelogenous leukemia [5]. BPDCN was once believed to be derived from natural killer cells and is now recognized as originating from precursors of plasmacytoid dendritic cells (DCs). DCs comprise two
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