CONCLUSION:
Relapsed/refractory BPCDN has limited therapeutic options and an overall dismal outcome. Conventional therapies have shown to be beneficial but transient remission forced to undergo allogeneic stem cell transplantation. Targeted therapy in combination with bcl-2 inhibitor molecule or demethylating agents are promising but rarity of the disease is an obstacle for prospective studies. Data for BPDCN treatment is extremely limited in pediatric age group. This is the first case report for combination molecular targeted therapies which can be safe and well tolerated options for difficult to treat, chemoresistant patients.