In 2008, the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues introduced a new category for myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1. Many of these cases present as a myeloproliferative neoplasm, usually with eosinophilia.
The 8p11 myeloproliferative syndrome (MPD) also known as stem cell leukemia-lymphoma syndrome (SCLL) is associated with translocations disrupting FGFR1. Imatinib, which induces durable responses in MPDs with PDGFRA and PDGFRB fusion genes, is inactive in patients with FGFR1 translocations.
Clinical Applications
- Chronic Myelogenous Leukemia and Myeloproliferative Neoplasms (CML/MPN)