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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*

Journal of Pediatric Hematology/Oncology: November 2009 - Volume 31 - Issue 11 - pp 879-883
doi: 10.1097/MPH.0b013e3181b83fd0
Clinical and Laboratory Observations

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.

Departments of *Pathology,

Pediatrics-Hematology and Oncology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX; and †Department of Pathology, Mount Sinai School of Medicine, New York, NY

This work was supported by Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX.

Reprints: Dolores López-Terrada, MD, PhD, Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin Street, MC 1-2261 Houston, TX 77030 (e-mail: dhterrad@TexasChildrensHospital.org).

Received for publication January 19, 2009

accepted July 18, 2009

The authors declare no competing financial interests.

© 2009 Lippincott Williams & Wilkins, Inc.