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One of three myeloproliferative lymphoid neoplasms associated with rearrangement of PDGFRA, PDGFRB and FGFR1. All result from formation of a fusion gene encoding an aberrant tyrosine kinase.
This disease is derived from a pluripotent hematopoieticstem cell. Patients can present as lymphoma with mainly lymph node involvement, or with myeloproliferative features, such as splenomegaly, AML or myeloid sarcoma.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9960/3.)
There is no established tyrosine kinase inhibitor therapy for MPN with FGFR1 rearrangement.
Interferon has induced a cytogenetic response in several patients. Until specific therapy is developed, hematopoieticstem cell transplantation should be considered even in those who present in chronic phase.