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Biphenotypic Hematologic Malignancy: A Case Report of the 8p11 Myeloproliferative Syndrome in a Child

Chen, Xiangli PhD*; Zhang, Yin MD*; Li, Yulong MSc*; Lei, Pingchong PhD*; Zhai, Yaping MD *; Liu, Linxiang MD

Journal of Pediatric Hematology/Oncology: August 2010 - Volume 32 - Issue 6 - pp 501-503
doi: 10.1097/MPH.0b013e3181e413fa
Clinical and Laboratory Observations

The 8p11 myeloproliferative syndrome, also known as stem cell leukemia/lymphoma, is a rare, atypical, myeloproliferative disorder and lymphoid malignancy associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand-independent FGFR activity. To date, 8 partner genes have been identified in association with FGFR1 rearrangements. The most frequent FGFR1 translocation partner is the zinc finger gene ZNF198 located at 13q11. Disease phenotypes associated with this translocation include poor prognosis and transformation to acute leukemia and non-Hodgkin lymphoma. In common with a T-cell phenotype, obtaining and maintaining remission is difficult by conventional chemotherapy. This study describes an illustrative case of 8p11 myeloproliferative syndrome/stem cell leukemia/lymphoma outlining its chief features and historical developments.

*Department of Clinical Hematology, Henan Provincial People's Hospital

Department of Clinical Hematology, First Hospital of Zhengzhou University, Zhengzhou, Henan Province, China

Reprints: Yin Zhang, MD, Department of Clinical Hematology, Henan Provincial People's Hospital, 7 Wei Wu Road, Zhengzhou 450003, Henan Province, China (e-mail: springcoming369@163.com).

Received for publication July 8, 2009; accepted April 6, 2010

© 2010 Lippincott Williams & Wilkins, Inc.