Clinical and Pathological Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm

Abstract

To explore the clinical manifestation, pathological features and treatment regimen of blastic plasmacytoid dendritic cell neoplasm (BPDCN). The clinical and pathologic features, diagnosis, treatment and prognosis of 5 BPDCN patients were analyzed retrospectively. 5 patients had skin lesions, and bone marrow involvement. The immunohistochemical staining showed that the CD56 expression was observed in 5 patients, CD4 and CD123 in 4 patients, and CD43 expressed in 3 patients. After initial treatment, 3 patients achieved complete remission and 1 patient did not. One patient showed disease progression after partial remission for 1 month. Two young patients underwent sibling allo-PBHSCT, and then achieved long-term survival. BPDCN often presents skin lesions as the first symptom, moreover, soft tissues, lymph nodes and bone marrow were involved, which suggested the disease progresses. There is currently no uniform treatment protocol for this disease. Active allogeneic peripheral blood hematopoietic stem cell transplantation in the CR1 phase for appropriate patients may increase their chances for long-term survival.