A 67-year-old man with cutaneous nodules was referred to a dermatologist, presenting with fatigue and weight loss. Physical examination revealed multiple purpuric nodules on the arms, legs, and torso. Laboratory results showed abnormal white blood cell count, hemoglobin level, and platelet count. Further tests confirmed the diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN).
During a discussion, possible differential diagnoses were suggested, including Sweet syndrome and angiosarcoma. The use of tagraxofusp (Elzonris) as frontline therapy was highlighted, with the patient achieving a complete response after one cycle of treatment. The high response rate of tagraxofusp in BPDCN was noted, although concerns were expressed over the adverse event profile, particularly capillary leak syndrome. The potential role of stem cell transplant as bridging therapy and post-transplant maintenance was also discussed.
However, in a case update, it was revealed that the patient declined allogeneic hematopoietic cell transplant and subsequently experienced relapse. Treatment options for relapsed or progressive BPDCN were explored, including chemotherapy, BCL2 antagonists, and participation in ongoing clinical trials investigating anti-CD123 therapies.
Overall, the article provides an overview of a case of BPDCN, discussing diagnostic workup, treatment options, and the importance of clinical trials in a disease where no standard treatment exists.
