eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2020-08-14 8 12 438 442 10.12691/ajmcr-8-12-1 AJMCR20208121 article Sruthi Roopa BM sruthiroopa18@gmail.com 1 Ramya Ananthakrishnan 1 AllwynYabesh T 1 Rameez Ahamed 1 Raja Thirumalairaj 1 Department of Medical Oncology, Apollo Cancer Institute, Chennai, India Blastic plasmacytoid dendritic cell neoplasm also formerly known as Blastic NK cell lymphoma is a very rare haematological malignancy. WHO defined BPDCN as a neoplasm with features of cutaneous lymphoma and/or leukaemia and designated BPDCN in a separate category under the myeloid class of neoplasms since 2008. Diagnosis of BPDCN requires both morphological evidence of plasmacytoid dendritic blast cells and immunohistochemical positivity for CD123, CD4, CD56 and TLC-1. Unlike other haematological malignancies there are no established optimal chemotherapy regimens for BPCDN although standard chemotherapeutic regimens used for induction treatment of AML, ALL and high grade lymphoma have shown complete remission rates albeit for a short duration, with ALL regimens having a higher remission percentage among both adults and children. Here we present a case of BPDCN without skin involvement. http://pubs.sciepub.com/ajmcr/8/12/1/ajmcr-8-12-1.pdf BPCDN pathological features Immunohistochemical criteria prognosis chemotherapy role