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Slobodnyuk et al. (Abstract)

Cellular Therapy and Transplantation (CTT), Vol. 3, No. 9
doi: 10.3205/ctt-2010-No9-abstract57
© The Authors. This abstract is provided under the following license: Creative Commons Attribution 3.0 Unported

Abstract accepted for "4th Raisa Gorbacheva Memorial Meeting on Hematopoietic Stem Cell Transplantation",
Saint Petersburg, Russia, September 18–20, 2010

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The case of blastic plasmacytoid dendritic cell neoplasm in a 51-year-old woman without cytogenetic abnormalities

Konstantin Yu. Slobodnyuk1, Vasily A. Shuvaev2, Ekaterina B. Rusanova1, Margarita V. Gorchakova1, Antonina V. Kurtova3, Denis V. Cherednichenko1, Yekaterina E. Zueva1

1Pavlov State Medical University, Saint-Petersburg, Russia; 2Russian Research Institute of Hematology and Transfusiology, Saint-Petersburg, Russia; 3Baylor College of Medicine, Houston, Texas, United States of America

Correspondence: Konstantin Yu. Slobodnyuk, Pavlov State Medical University, 6/8, Tolstoy str., Saint-Petersburg, 199044, Russia, E-mail: k.slobodnyuk@spam is badgmail.com


Background. Blastic plasmacytoid dendritic cell leukemia (PDCL) or agranular CD4+CD56+ neoplasm is a very rare disease that was revised in the WHO classification in 2008. The cells of PDCL are derived from precursors of a subset of dendritic cells – plasmacytoid dendritic cells – and thus PDCL is a myeloid-related neoplasm. The clinical course of malignant growth is aggressive and it is characterized by multiple hematodermic lesions and may attend a regional lymphadenopathy.

Aims. The aim of this study is to demonstrate the immunophenotypical features of the tumor cells of PDCL.

Methods. A 51-year-old Russian female presented in January of 2009 with skin lesions on her head. The results of the first skin biopsy diagnosed unspecified T cell lymphoma. After four cycles of CHOP chemotherapy hematodermic remission lasted for four months. A second pathology investigation of the skin lesions, occipital, axillary, and inguinal lymph nodes at relapse established myeloid sarcoma. A bone marrow biopsy showed an almost 87% population of blast cells. Five-color flow cytometry and chromosomal analysis were performed on the bone marrow aspirate.

Results. The tumor cells have blast-like/plasmacytoid morphology. Neoplastic cells expressed CD2, CD4, CD45 (dim), CD56 with co-expression of CD7, CD10, and HLA-DR. Other markers associated with T, B, and myeloid cells, and monocytes are negative. Thus the immunophenotype of the blasts is CD45dimCD4+CD56+CD2+ with co-expression of CD7 and CD10. A cytogenetic study did not show any clonal chromosomal abnormalities.

Conclusions. All of these features are consistent with the PDCL neoplasm, a distinctive entity in the updated 2008 WHO classification.

Keywords: acute myeloid leukemia, dendritic cell, flow cytometry, cytogenetics


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