LQCL2408 - CMLE - Blastic Plasmacytoid Dendritic Cell Neoplasm-Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic Plasmacytoid Dendritic Cell Neoplasm

Pdf Summary

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic neoplasm with a challenging diagnosis. Patients often present with skin lesions, bone marrow involvement, and systemic manifestations. The disease predominantly affects older adults and has a male predominance. Pathognomonic features include atypical cells with distinct morphological and immunophenotypic characteristics, such as fine chromatin, CD123 positivity, and absence of specific markers like CD34. Diagnosis involves meticulous examination and comprehensive immunophenotyping. Treatment typically involves chemotherapy regimens, with stem cell transplants showing promise for prolonged survival. The disease has a poor prognosis, with relapses being common despite initial treatment responses. Recognition of specific BPDCN cell features, such as pseudopodia and "string of pearls," is crucial for timely diagnosis and management. Research on genetic mutations associated with BPDCN is ongoing. Targeted therapies like tagraxofusp have shown improved outcomes in conjunction with standard treatments. Overall, early identification and understanding of BPDCN characteristics are vital for prompt intervention and patient care.

Keywords

Blastic plasmacytoid dendritic cell neoplasm

BPDCN

Hematologic neoplasm

Skin lesions

Bone marrow involvement

CD123 positivity

Stem cell transplants

Poor prognosis

Genetic mutations

Tagraxofusp