[Clinical and Pathological Features of Blastic Plasmacytoid Dendritic Cell Neoplasm].

Objective: To summarize and analyze the clinical features of blastic plasmacytoid dendritic cell neoplasm (BPDCN), so as to enhance the understanding of this disease.

Methods: The clinical manifestations, immunophenotype, pathological features, treatment and prognosis of 11 cases of BPDCN were retrospectively analyzed.

Results: Among the 11 patients diagnosed with BPDCN, there were 8 males and 3 females, with a median age of 44 (6-81) years. The main clinical symptoms were rash and mass, accompanied by lymph node and bone marrow involvement. The neoplastic plasmacytoid dendritic cells (pDC) were positive for HLA-DR, CD4, CD56 and CD123, but negative for cCD3, cMPO and cCD79a; In some cases, they were also positive for CD38, CD99 and CD36. Patients who have underwent surgical resection and those who experienced multiple chemotherapy failures tend to have rapid recurrence and shorter survival time. Patients who achieved complete remission (CR) after the first chemotherapy exhibit no expression of CD56 on pDC cells, and tend to have a longer survival time after bone marrow transplantation.

Conclusion: The immunophenotype of BPDCN is heterogeneous. CD56 is a reliable marker to distinguish neoplastic pDC cells from reactive pDC cells. The BPDCN patients who underwent hematopoietic stem cell transplantation (HSCT) after achieving remission from initial chemotherapy tend to have a better prognosis.