Korean J Intern Med.  2017 Sep;32(5):890-899. 10.3904/kjim.2015.406.

Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell neoplasm: a single-center experience in Korea

Affiliations
  • 1Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. kstwoh@skku.edu
  • 2Division of Hematology-Oncology, Department of Medicine, Veterans Health Service Medical Center, Seoul, Korea.
  • 3Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 4Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 5Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 6Department of Health Sciences and Technology, Samsung Advanced Institute for Health Sciences & Technology, Sungkyunkwan University, Seoul, Korea.

Abstract

BACKGROUND/AIMS
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal.
METHODS
We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014.
RESULTS
Ten patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven patients were male. Sites of disease involvement were the skin (n = 7), lymph node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue (n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin, L-asparaginase) were used as a first-line treatment. Although all patients treated with intensified chemotherapy showed an objective response (five patients with complete response) with median progression-free survival of 11.2 months (range 6.2 to 19.4), complete remission was not sustained for more than 2 years in any case. The response was relatively long-lived compared with previously reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like regimens, but the above regimens do not result in long-term remission.
CONCLUSIONS
All patients treated with hyperCVAD or VPDL showed an objective response, but the duration of response was relatively short. Thus, the development of more effective induction as well as consolidation treatment strategy should be warranted to improve this rare disease entity.

Keyword

Blastic plasmacytoid dendritic cell neoplasm; Therapeutics; Survival outcome

MeSH Terms

Bone Marrow
Cyclophosphamide
Daunorubicin
Dendritic Cells*
Dexamethasone
Diagnosis
Disease-Free Survival
Doxorubicin
Drug Therapy
Hematologic Neoplasms
Humans
Korea*
Liver
Lymph Nodes
Male
Methotrexate
Methylprednisolone
Prognosis
Rare Diseases
Retrospective Studies
Skin
Skin Manifestations
Spleen
Vincristine
Cyclophosphamide
Daunorubicin
Dexamethasone
Doxorubicin
Methotrexate
Methylprednisolone
Vincristine
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