Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

Ozdemir Z. N., CENGİZ SEVAL G., Sahin U., Uslu A., Gunduz M., CİVRİZ BOZDAĞ S., ...Daha Fazla

INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION, cilt.37, sa.1, ss.67-75, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 37 Sayı: 1
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1007/s12288-020-01313-9
  • Dergi Adı: INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE
  • Sayfa Sayıları: ss.67-75
  • Anahtar Kelimeler: Blastic plasmacytoid dendritic cell neoplasm, Clinical and biological features, Intensive chemotherapy regimens, Allogeneic hematopoietic stem cell transplantation, LEUKEMIC PRESENTATION, DIAGNOSIS, MANIFESTATIONS, INVOLVEMENT, FEATURES, SERIES
  • Ankara Üniversitesi Adresli: Evet

Özet

Purpose Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients. Methods Nine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated. Results All patients (n = 9) were male, median age was 64 (21-80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3-48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7-19.1) months. Conclusion Intensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease.