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ctt-journal > Gindina et al. (Abstract)

Gindina et al. (Abstract)

Cellular Therapy and Transplantation (CTT), Vol. 3, No. 12
doi: 10.3205/ctt-2011-No12-abstract19

© The Authors. This abstract is provided under the following license: Creative Commons Attribution 3.0 Unported

Abstract accepted for "5th Raisa Gorbacheva Memorial Meeting Hematopoietic Stem Cell Transplantation in Children and Adults", Saint Petersburg, Russia, September 18–20, 2011

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A new case of the 8p11 myeloproliferative syndrome successfully treated by allogeneic unrelated bone marrow transplantation

Tatiana L. Gindina, Sergey N. Bondarenko, Olga A. Slesartchuk, Maria E. Vlasova, Elena S. Nikolaeva, Nikolay N. Mamaev

R.M. Gorbacheva Memorial Institute of Children Hematology and Transplantation, St. Petersburg Pavlov State Medical University, St. Petersburg, Russia

Correspondence: Tatiana L. Gindina, R.M. Gorbacheva Memorial Institute of Children Hematology and Transplantation, St. Petersburg, St. Petersburg Pavlov State Medical University, 6/8 Tolstoy str., St. Petersburg 197022, Russia, E-mail: tatgindina@spam is badgmail.com

Abstract

The 8p11 myeloproliferative syndrome (EMS), also called stem cell leukemia/lymphoma (SCLL), is a rare disease that is characterized by rapid progression to acute myeloid leukemia and poor response to treatment. The only curative approach is allogeneic hematopoietic stem cell transplantation. Non-random cytogenetic abnormality is a translocation at the 8p11 locus, which involves the fibroblast growth factor 1 (FGFR1) gene.

A new case of EMS/SCLL in a 19-year-old female is presented, which was diagnosed in July 2009. According to clinical and laboratory parameters this was a typical form of the disease, e.g., association of a bcr/abl-negative myeloproliferative disorder and a T-cell lymphoblastic lymphoma. WBC at diagnosis was 375.0 x109/l. Bone marrow was hypercellular with myeloid hyperplasia, and contained 8% blasts. Erythropoiesis was decreased; megakaryocytes had dysplastic features. A lymph node biopsy showed T-cell lymphoma with CD2, CD3, CD5, TdT-positivity, and CD34-negativity. The Ki-67 antigen was positive in 50% of neoplastic cells. Cytogenetic study revealed a translocation t(6;8)(q27;p11) which has been confirmed by mFISH. The allogeneic unrelated bone marrow transplantation was carried out in March 2011, which allowed the achievement of complete clinical and cytogenetic remissions, evidenced serially.

Keywords: 8p11 myeloproliferative syndrome, stem cell leukemia/lymphoma, allogeneic BMT