Clinical Analysis On Four Cases Of Blastic Plasmacytoid Dendritic Cell Neoplasm And The Related Literature Review

Objective: To summarize the essentials of diagnosis,therapy and prognosis of blastic plasmacytoid dendritic cell neoplasm,and to improve the recognition of BPDCN for clinical doctors,and to provide the basis for further diagnosis,therapy and prognosis.Methods: The data of four BPDCN patients who were diagnosised and treated in department of hematology of Shanxi Cancer Hospital were recruited,such as general information,clinical features,histopathologic results,immunephenotype,other accessory examination results,theropy and prognosis.The author retrospectively analyzed the data,and reviewed the related literature.Results: Among the four cases of blastic plasmacytoid dendritic cell neoplasm,three were females and one was male.Their age were respectively 66,15,63 and 59 years old,and Their median age was 50.8 year.The initial symptoms of the four cases were skin lesions,one of which was only involved in skin,and another three were involved in skin,bone marrow and lymph nodes.In the three cases,two of which were involved in central nervous system.The skin biopsies of three case showed pleomorphic cells of intermediate-size in dermis area,but no tumor cell in epidermis.The lymph node biopsy of one patient showed that the normal structure of the lymph node disappeared,and insteaded of intermediate-size lymphoid cells,and there was no vasal necrosis and invasion.All cases expressed CD123,CD56 and CD4,but MPO,CD20,CD3,CD79 a,CD30 and EBER were negative.One patient withdrawn therapy and was lost to follow up.And another three patients received chemotherapy,and their early cure effect were better,but were relapsed rapidly,two of which were died of central nervous system involment,whose mean survival time were 12.5 months,and the other one patient was treating.Conclusion: BPDCN is a rare disease with specific immunophenotypes.It is common for elderly males,and frequently present as cutaneous involvement initially,and may be easily misdiagnosed.It is highly aggressive and progresses rapidly,with a poor prognosis.The diagnosis of BPDCN should be combined clinical manifestation with morphology and immunophenotyping.The disease incidence is low,and there is no unified standard of treatment.