Case Report

Blastic Plasmacytoid Dendritic Cell Neoplasm Expressing the CD13 Myeloid Antigen
Daichi Inoue^{a, e}, Kyoko Maruyama^d, Kazunari Aoki^a, Seiji Nagano^a, Hayato Maruoka^b, Yukihiro Imai^c, Kiminari Ito^d, Takayuki Ishikawa^a, Takayuki Takahashi<sup>a

a</sup>Department of Hematology and Clinical Immunology,
^bClinical Laboratory,
^cDepartment of Clinical Pathology, Kobe City Medical Center General Hospital,
^dDepartment of Cell Therapy, Foundation of Biomedical Research and Innovation, Kobe, and
^eDivision of Cellular Therapy, Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan

Address of Corresponding Author

Acta Haematol 2011;126:122-128 (DOI: 10.1159/000328180)

  Key Words

• Antigen-presenting cells
• Blastic plasmacytoid dendritic cell neoplasm
• CD13
• CD40 ligand
• Dendritic cells
• IL-3

  Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), currently considered to originate from immature plasmacytoid dendritic cells (DC), is a rare and aggressive CD4+CD56+ neoplasm that frequently involves the skin and bone marrow. We present a case of an 80-year-old man with a CD4+CD56+ BPDCN that affected the orbital cavity and bone marrow. Although BPDCN has not been reported to express any lineage-specific markers, the neoplastic cells strongly expressed the CD13 antigen. Therefore, in addition to pathological examination, we attempted to induce in vitro morphological and surface marker changes with IL-3 and CD40 ligand. After treatment with these cytokines, the tumor cells enlarged markedly, acquired many fine dendrites, similar to mature DC, and showed enhanced expression of antigens specific to DC or antigen-presenting cells, such as CD40, CD80, CD83 and CD86. To the best of our knowledge, this is the first report of BPDCN expressing a myeloid antigen, CD13, although CD33 expression has been described in some cases. The present patient received 2 courses of combination chemotherapy consisting of cytarabine and etoposide, which resulted in complete remission. Given that the cellular origin of plasmacytoid DC is still controversial, myeloid antigen expression involving CD13 may not exclude a diagnosis of BPDCN.

Copyright © 2011 S. Karger AG, Basel

  Author Contacts

Daichi Inoue
Department of Hematology and Clinical Immunology
Kobe City Medical Center General Hospital
4-6 Minatojima-Nakamachi, Chuo-ku, Kobe 650-0046 (Japan)
Tel. +81 78 302 4321, E-Mail daichi-i@hotmail.co.jp

  Article Information

Received: February 7, 2011
Accepted after revision: April 5, 2011
Published online: June 24, 2011
Number of Print Pages : 7
Number of Figures : 3, Number of Tables : 1, Number of References : 21