Blastic plasmacytoid dendritic cell neoplasm (BPDCN) — diagnostic and therapeutic challenge

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy. The disease usually affects the skin, bone marrow, peripheral blood and less commonly lymph nodes. The actual incidence of BPDCN is currently unknown. The data presented in the literature most often relate to one cases or small groups of patients, rarely they are multicentre studies. Diagnosis of BPDCN is based on histopathological examination and immunohistochemical stains. The diversity of the clinical manifestations and the PBDCN immunophenotype is the cause of significant difficulties in making a diagnosis and can lead to diagnostic errors. The optimal treatment for patients with this cancer has not yet been established. Responses to various chemotherapy regimens are unsatisfactory. Recent literature has reported that bone marrow allograft or targeted therapy may improve treatment outcomes in this group of patients. The paper presents the case of a 75-year-old man with BPDCN diagnosis. Attention was paid to diagnostic difficulties in patients with BPDCN and the differentiation of this rare disease with other hematological malignancies was discussed. The need for a national register of BPDCN patients has been highlighted. This could contribute to expanding knowledge about this cancer and to the development of effective, standard therapeutic treatment.