A Very Rare Type of Leukemia in a Young Pediatric Patient Treated with Hematopoietic Stem Cell Transplantation: Blastic Plasmacytoid Dendritic Cell Neoplasm

Tekkeşin F., Asarcikli F., Yildirim Ü. M., Zemheri I. E., Kiliç S. Ç.

Journal of Pediatric Hematology/Oncology, 2020 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2020
  • Doi Numarası: 10.1097/mph.0000000000001999
  • Dergi Adı: Journal of Pediatric Hematology/Oncology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE
  • Anahtar Kelimeler: blastic plasmacytoid dendritic cell neoplasm, hematopoeitic stem cell transplantation, CD4/CD56/CD123, pediatric
  • İstanbul Üniversitesi Adresli: Hayır

Özet

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly identified, very rare, highly aggressive hematopoietic neoplasm, primarily found in elderly males. They typically present in the form of skin involvement with a high frequency of lymph node and bone marrow involvement. BPDCN has a very poor prognosis, with no consensus on a widely accepted treatment modality. Here we present a very young patient with BPDCN, who presented with generalized lymphadenopathy, skin involvement, and leukemic blasts in the bone marrow. She was treated with high-risk acute lymphocytic leukemia protocol, followed by allogeneic hematopoietic stem-cell transplantation, and has been in clinical remission for 12 months.