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Multilineage involvement of the fusion gene in patients with FIP1L1/PDGFRA-positive HES1/17/2006
British Journal of Haematology
Volume 132 Page 286  - February 2006
doi:10.1111/j.1365-2141.2005.05863.x
  Volume 132 Issue 3  
Multilineage involvement of the fusion gene in patients with FIP1L1/PDGFRA-positive hypereosinophilic syndrome
Jamie Robyn1, Steven Lemery2, J. Philip McCoy3, Joseph Kubofcik4, Yae-Jean Kim4, Svetlana Pack5, Thomas B. Nutman4, Cynthia Dunbar2 and Amy D. Klion4
 Summary

Myeloproliferative hypereosinophilic syndrome (MHES) is a disorder characterised by male predominance, marked eosinophilia, splenomegaly, tissue fibrosis, elevated serum tryptase and the presence of the FIP1L1/PDGFRA fusion gene in peripheral blood mononuclear cells. The characteristic hypercellular bone marrow with dysplastic eosinophils and spindle-shaped mast cells suggest that multiple lineages may be involved in the clonal process. To determine which haematopoietic lineages are involved in MHES, we purified cells of specific lineages from patients with MHES and used nested reverse transcription polymerase chain reaction (RT-PCR), quantitative RT-PCR and fluorescence in situ hybridisation to analyse the purified cell populations for the presence of the fusion gene. The fusion gene was detected in eosinophils, neutrophils, mast cells, T cells, B cells and monocytes. These results suggest that the mutation arises in a pluripotential haematopoietic progenitor cell capable of giving rise to multiple lineages. The basis for the preferential expansion of eosinophils and mast cells remains unclear.

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