Diagnostic challenge in a case of blastic plasmacytoid dendritic cell neoplasm; a rare entity

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive haematological malignancy. Though the criteria for diagnosis is well defined by a set of characteristic features including clinical presentation, morphology and immunophenotype, the rarity of the disease, lack of awareness, heterogenous initial clinical presentation and lack of recurrent specific chromosomal abnormalities, make early diagnosis difficult. A high index of suspicion in appropriate cases is useful in arriving at the correct diagnosis. In young patients, intensive chemotherapy followed by consolidation with allogenic haematopoietic stem cell transplantation is considered the most effective treatment strategy. Recently, targeted therapies have shown promising results.