Blastic Plasmacytoid Dendritic Cell Neoplasm in an Adolescent Managed in a Resource-Limited Setting: A Case Report

Area of Science:

• Hematology
• Oncology
• Genetics

Background:

• Blastic plasmacytoid dendritic cell neoplasms (BPDCN) are rare, aggressive hematologic malignancies.
• BPDCN are infrequently diagnosed in adolescents and present diagnostic challenges.

Purpose of the Study:

• To report a rare case of BPDCN in a 16-year-old male.
• To highlight diagnostic and therapeutic challenges in resource-limited settings.

Main Methods:

• Case report of a 16-year-old male with BPDCN.
• Diagnostic workup included bone marrow aspirate, flow cytometry, lymph node biopsy, and peripheral blood next-generation sequencing (NGS).

Main Results:

• The patient presented with lymphadenopathy, pancytopenia, and constitutional symptoms.
• Bone marrow aspirate showed 84% blasts; NGS revealed an NRAS p.(G12R) mutation.
• The patient received AML and ALL-type chemotherapy and died from neutropenic sepsis.

Conclusions:

• BPDCN diagnosis and treatment are challenging, especially in adolescents and resource-limited settings.
• The NRAS mutation may play a role in BPDCN pathogenesis.
• Early diagnosis and targeted therapy are crucial for improving outcomes in BPDCN.