Molecular monitoring of 8p11 myeloproliferative syndrome in an infant

Wenyong W. Zhang, Sultan Habeebu, Andrea M. Sheehan, Rizwan Naeem, Vivian S. Hernandez, Zoann E. Dreyer, Dolores López-Terrada

Research output: Contribution to journalArticle

9 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)879-883
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume31
Issue number11
DOIs
Publication statusPublished - Nov 1 2009
Externally publishedYes

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Keywords

  • EMS syndrome
  • Minimal disease monitoring
  • Molecular

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Zhang, W. W., Habeebu, S., Sheehan, A. M., Naeem, R., Hernandez, V. S., Dreyer, Z. E., & López-Terrada, D. (2009). Molecular monitoring of 8p11 myeloproliferative syndrome in an infant. Journal of Pediatric Hematology/Oncology, 31(11), 879-883. https://doi.org/10.1097/MPH.0b013e3181b83fd0