Plasmacytoid dendritic cell leukemia in children.


CD4/CD56 malignancies are rare hematologic neoplasms, which have recently been shown to represent the malignant counterpart of plasmacytoid dendritic cells (pDC). A 5-year-old boy initially presented with multiple subcutaneous lesions on his upper and lower extremities. Skin biopsy results showed large atypical lymphoid cells in the dermis. The blast cells were stained with CD4 and CD56. In the bone marrow aspirate, 20% of the blast cells were found. The patient was diagnosed as acute unclassified leukemia and received chemotherapy designed for the treatment of acute myeloid leukemia. He achieved a complete remission that lasted for 8 months. However, multiple subcutaneous lesions recurred 1 month after the end of the therapy, with increasing blast cells in his blood. Immunophenotypically, the blast cells were positive for CD2, CD4, CD7, and CD56, and negative for CD3, CD13, CD19, CD33, and CD34 antigens. The blast cells were positive for CD123 (interleukin-3 receptor alpha chain) and blood dendritic cell antigen-2, which are expressed on pDC. The patient was diagnosed as acute leukemia derived from pDC. The CD4, CD56, CD3, CD13, CD19, CD33 profile is highly suggestive of this disease, and the CD123 and blood dendritic cell antigen-2 markers are useful in helping to diagnose pDC leukemia.

DOI: 10.1097/MPH.0b013e31819b7215

Cite this paper

@article{Hama2009PlasmacytoidDC, title={Plasmacytoid dendritic cell leukemia in children.}, author={Asahito Hama and K Kudo and Bustos Villalobos Itzel and Hideki Muramatsu and Nobuhiro Nishio and Nao Yoshida and Yoshiyuki Takahashi and Hiroshi Yagasaki and Masafumi Ito and Seiji Kojima}, journal={Journal of pediatric hematology/oncology}, year={2009}, volume={31 5}, pages={339-43} }