Work

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

Year: 2024

Type: article

Abstract: 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.

Source: The Sri Lanka Journal of Haematology

DOI: 10.4038/tsljh.v16i1.22

Authors: T. Sooriyakumar, Y. Natkunam, G. Selvaratnam

Institutions: Jaffna Teaching Hospital, University of Jaffna, Stanford Medicine, Stanford University

Language: English

Topic: Histiocytic Disorders and Treatments

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

OpenAlex ID: W4405821748