Progress In Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm(BPDCN) is a rare hematological malignancy with an aggressive clinical course,accounted for 0.44% of blood tumor and 0.7% of skin lymphoma. Most patients with BPDCN have skin lesions and simultaneous involvement of the peripheral blood, bone marrow, and lymph nodes. Samples from patients have been confirmed that BPDCN was originated in the abnormalplasmacytoid dendritic cells(p DCs). The diagnosis of BPDCN is based on the characteristic morphocytology and immunophenotype of malignant cells,p DCs mainly expressing CD4,CD56, CD123, TCL, blood dendritic cell antigens 2 and 4(CD303 and CD304), and CD2 AP markers. Multiple chromosomal abnormalities and gene mutations previously reported in patients with myeloid and selected lymphoid neoplasms were identified in approximately 60% of patients with BPDCN. Prospectively controlled studies to guide treatment decisions are lacking. The overall response rate with aggressive acute lymphoblastic leukemia–type induction regimens was as high as 90%, but the durability of response was short. Median survival rates ranged between 12 and 14 months. Although BPDCN is initially sensitive to conventional chemotherapy regimens, this response is relatively short and long-term prognosis is poor.Allo-geneic hematopoietic stem cell transplantation, particularly when performed during the first remission, may produce durable remissions in selected adults. Patients with relapsed disease may respond to L-asparaginase–containing regimens. Radiation therapy is used in some cases(isolated lesions, elderly patients, or patients with comorbidities). There is no standardized treatment.In the near future, novel targeted therapies perhaps improve the outcomes for patients with BPDCN.