TY - JOUR
T1 - Plasmacytoid dendritic cell leukemia in children
AU - Hama, Asahito
AU - Kudo, Kazuko
AU - Itzel, Bustos Villalobos
AU - Muramatsu, Hideki
AU - Nishio, Nobuhiro
AU - Yoshida, Nao
AU - Takahashi, Yoshiyuki
AU - Yagasaki, Hiroshi
AU - Ito, Masafumi
AU - Kojima, Seiji
N1 - Copyright:
Copyright 2012 Elsevier B.V., All rights reserved.
PY - 2009/5
Y1 - 2009/5
N2 - 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 α 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.
AB - 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 α 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.
UR - http://www.scopus.com/inward/record.url?scp=66149143597&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=66149143597&partnerID=8YFLogxK
U2 - 10.1097/MPH.0b013e31819b7215
DO - 10.1097/MPH.0b013e31819b7215
M3 - Article
C2 - 19415013
AN - SCOPUS:66149143597
VL - 31
SP - 339
EP - 343
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
SN - 1077-4114
IS - 5
ER -