FGFR1 rearrangements (8p11)
Myeloproliferative disorder in association with an aggressive lymphoproliferative disorder is a rather unusual phenomenon. Translocations affecting FGFR1 gene (8p11) are rare events that have been reported in acute myeloid leukemia and myeloproliferative neoplasms that suggest a bilinear differentiation from a pluripotent stem cell. The 8p11 myeloproliferative syndrome (EMS), also known as ‘stem cell’ lymphoma-leukemia syndrome (SCLL) is a relatively rare condition characterized by BCR/ABL negative myeloproliferative disease and lymphoma, normally lymphoblastic lymphoma (T-LBL). As in chronic myelogenous leukemia (CML), the chronic myeloproliferative phase becomes myeloblastic, typically after a year of diagnosis.
Acute leukemia shows resistance to conventional chemotherapy. Imatinib, which induces a lasting response in myeloproliferative disorders (MPSs) PDGFRα and PDGFRβ translocations, is not effective in the treatment of neoplasms with FGFR1 rearrangements.
References:
- Chase et al. (2007) Blood, 110, 3729-3734.
- Walz et al. (2005) Leukemia, 19, 1005-1009.
- Cross and Reiter (2002) Leukemia, 16, 1207-1212.
