January 23rd of 2025 marks the International BPDCN Day. We unite to shed light on Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN), a rare and aggressive hematologic malignancy that often remains underdiagnosed due to its rarity and complex presentation.
In 2008, BPDCN was recognized by the WHO as a distinct entity and separately listed in the group of acute myeloid leukemias and related precursor neoplasms. BPDCN predominantly affects the skin, bone marrow, and lymph nodes, posing significant diagnostic and therapeutic challenges. Despite advances in hematology, much remains to be understood about its pathogenesis, optimal treatment strategies, and long-term outcomes. Greater awareness and focused research are critical to improving the prognosis for patients worldwide.
At present, there is no consensus on the optimal treatment of BPDCN. The majority of patients receive multi-agent chemotherapy with AML or ALL treatment regimens, while a few patients undergo allogeneic hematopoietic stem cell transplantation (HSCT). Different novel and innovative therapies are in development to target surface molecules in BPDCN. However, patients are still in need of better treatments and the optimal therapy for the disease remains to be determined.
Unfortunately, projects devoted to this mission are not many either. The BPDCN International Registry is one of them, aimed in building a large database of patients to investigate the characteristics and outcomes of the disease with different treatment regimens, evaluate prognostic factors, and generate data-based prospective treatment recommendations.
Let’s unite on International BPDCN Day to amplify our collective efforts, inspire new collaborations, and give hope to patients and their families. Together, we can make a meaningful difference in the fight against this disease.
