Genomic Aberrations Involving 12p/ETV6 Are Highly Prevalent in Blastic Plasmacytoid Dendritic Cell Neoplasms and Might Represent Early Clonal Events

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic neoplasm derived from plasmacytoid dendritic cells. Skin lesions are the most common presenting clinical feature, seen in nearly 85% of patients. While some patients may present with disease localized to the skin, most present with or eventually develop systemic involvement most commonly involving the bone marrow.[1 –3] Patients have a median survival of 12 months, and to date therapeutic options remain limited in scope and effectiveness, with the only curative option being stem cell transplant (SCT) therapy in eligible patients.[4] Most patients are induced with acute lymphoblastic leukemia (ALL)-type regime ns, although some novel therapeutic modalities, such as the BCL2 inhibitor venetoclax and the interleukin-3 receptor (IL3R) inhibitor SL-401, have shown potential benefits.[5–7]
Source: Leukemia Research - Category: Hematology Authors: Tags: Research paper Source Type: research