Blastic plasmacytoid dendritic cell neoplasm in a child: A case report of this rare entity

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children. It usually manifests with skin lesions on face, scalp or trunk with frequent involvement of peripheral blood, bone marrow and lymph nodes. Due to rarity and wide clinical spectrum, these cases are difficult to diagnose and require extensive immunophenotypic workup. It is only recently recognized as a distinct clinico pathological entity and literature pertaining to pediatric experience is limited. We report a case of BPDCN in an eleven years old boy presented with skin nodules on cheek and forehaed. This case is reported to improve the knowledge of physicians regarding this rare disease. Diagnosis was established by bone marrow biopsy and flowcytometry that revealed expression of BPDCN’s pathognomonic markers CD4, CD56 and CD123. Standard treatment has not been defined to date however acute lymphoblastic leukemia type chemotherapy followed by stem cell transplant showed some optimistic results in children. Owing to our limited resources and poor prognosis of the disease with low overall survival rates patient was kept on palliation.

Keywords

• Blastic plasmacytoid dendritic cell neoplasms
• Cutaneous lesions
• Immunophenotyping
• NK cell leukemia
• CD56
• CD123