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One of three myeloproliferative and lymphoidneoplasms associated with rearrangement of PDGFRA, PDGFRB and FGFR1. All result from formation of a fusion gene encoding an aberrant tyrosinekinase.
This disease occurs in association with rearrangement of PDGFRB at 5q31-33. Usually there is (5;12)(q31-33;p12) with formation of an ETV6-PDGFRB fusion gene.
The features are most consistent with CMML, but some patients have been characterized as atypical chronic myeloidleukemia.