Blastic plasmacytoid dendritic cell neoplasm: A single centre Indian experience

TL;DR

This paper discusses the challenges of diagnosing and treating a rare blood cancer called BPDCN, based on experiences from an Indian hospital.

Contribution

The study highlights the management of three BPDCN cases, emphasizing the lack of effective therapies and diagnostic difficulties in this rare malignancy.

Findings

BPDCN presents diagnostic and therapeutic challenges due to its rarity and complex nature.

Current treatment strategies include ALL-like or AML-like therapies, especially for older adults.

The study underscores the need for more effective and standardized treatment approaches for BPDCN.

Abstract

BPDCN is an uncommon haematological malignancy recognised as a distinct entity by the WHO in 2016. It typically presents with skin lesions and involves bone marrow, lymph nodes, and CNS, affecting all age groups. The disease poses significant diagnostic challenges, with limited data on optimal treatment strategies, further contributing to its dismal prognosis. Standard regimens include ALL-like or AML-like therapies, particularly in older adults. We outline our experiences managing three consecutive BPDCN patients, emphasising the unique challenges oncologists face when treating this malignancy. This underscores scarcity of effective therapies, limited expertise, and intricacies involved in managing such a complex disease.