Blastic plasmacytoid dendritic cell neoplasm transformed from essential thrombocythemia with leukemic picture and no skin involvement – A case study

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare and fatal condition, which usually affects elderly males. It presents in the skin with frequent involvement of the bone-marrow, peripheral blood and lymph nodes. It has a dismal prognosis, with most patients dying within one year. The diagnosis is frequently challenging, with a characteristic immunophenotype. The best modality of treatment remains to be defined. Generally, patients require acute leukaemialike induction chemotherapy regimen. Here we present a local case who transformed from essential thrombocythemia into BPDCN over a 20-year period with sudden leukemic picture, and interestingly without any typical skin lesions.