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Int J Clin Exp Pathol 2013;6(11):2603-2608

Case Report
Sustained complete remission of a limited-stage blastic plasmacytoid dendritic cell
neoplasm followed by a simultaneous combination of low-dose DeVIC therapy and
radiation therapy: a case report and review of the literature

Kei-Ji Sugimoto, Asami Shimada, Nanae Yamaguchi, Hidenori Imai, Mutsumi Wakabayashi, Yasunobu Sekiguchi, Hiroshi Izumi, Yasunori Ota,
Norio Komatsu, Masaaki Noguchi

Department of Hematology, Juntendo University Urayasu Hospital, Urayasu, Japan; Department of Hematology, Juntendo University School of
Medicine, Tokyo, Japan; Department of Pathology, Juntendo University Urayasu Hospital, Urayasu, Japan; Department of Pathology, Research
Hospital, The Institute of Medical Science, The University of Tokyo, Japan

Received September 2, 2013; Accepted September 25, 2013; Epub October 15, 2013; Published November 1, 2013

Abstract: The patient was a 74-year-old man who was found to have a cutaneous mass on his left shoulder in February 2012. Because the
mass bled easily and was tending to grow, total resection of the cutaneous tumor, which measured approximately 5 cm x 3 cm, was performed
in July. Histopathological examination revealed a tumor that extended from the dermis to the cutaneous adipose tissue, but no invasion of the
epidermis was seen. The tumor cells were plasmacytoid cells ranging in size from small to intermediate, and there was no nuclear irregularity.
They had a high nuclear-cytoplasmic ratio, and nucleoli were observed. The tumor cells were CD4-positive, CD56-positive, and
CD123-positive, and they were AE1/AE3-negative, CD3-negative, CD20-negative, and myeloperoxidase-negative. 18F-fluorodeoxyglucose
positron emission tomography-computed tomography (FDG-PET/CT), a bone marrow examination, etc., were performed, but no lesions were
detected at other sites. Based on the above findings a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN), Stage IEA, was
made. Because the patient had limited-stage BPDCN and was elderly, we treated him with a simultaneous combination of low-dose DeVIC
(dexamethasone, VP16, ifosfamide, and carboplatin) therapy and local radiation therapy (LRT) and sustained a complete remission for
approximately 1 year. Simultaneous combination of non-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy
and LRT appeared to be useful in the treatment of limited-stage BPDCN even in the elderly. (IJCEP1309009).

Keywords: Blastic plasmacytoid dendritic cell neoplasm, DeVIC, local radiation therapy, CD4, CD56, CD123

Address correspondence to: Dr. Kei-Ji Sugimoto, Department of Hematology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu
279-0021, Japan. Tel: +81-47-353-3111; Fax: +81-47-381-5054; E-mail: keijis@juntendo.ac.jp