Skip Navigation LinksHome > November 2009 - Volume 31 - Issue 11 > Molecular Monitoring of 8p11 Myeloproliferative Syndrome in...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3181b83fd0
Clinical and Laboratory Observations

Molecular Monitoring of 8p11 Myeloproliferative Syndrome in an Infant

Zhang, Wenyong W. MD, PhD*†; Habeebu, Sultan MD, PhD*; Sheehan, Andrea M. MD*; Naeem, Rizwan PhD; Hernandez, Vivian S. MS*; Dreyer, ZoAnn E. MD; López-Terrada, Dolores MD, PhD*

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Abstract

The 8p11 myeloproliferative syndrome is a rare hematologic malignancy derived from a pluripotent hematopoietic stem cell associated with rearrangements involving the fibroblast growth factor receptor 1 (FGFR1) gene located on chromosome 8p11. The most common translocation, t(8;13) (p11;q13), results in a ZNF198-FGFR1 fusion gene and constitutively active FGFR1 tyrosine kinase activity. Typical pathologic findings include myeloid hyperplasia, lymphadenopathy, precursor T-lymphoblastic lymphoma, and eosinophilia. The disease is usually associated with an aggressive course and progression to acute myeloid leukemia is frequent. We report here the first case of 8p11 myeloproliferative syndrome in an infant and demonstrate the value of molecular testing in the diagnosis and minimal disease monitoring of this rare disease.

© 2009 Lippincott Williams & Wilkins, Inc.

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