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Code grade specified by pathologist. If no grade specified, code 9
Chronic eosinophilic leukemia with FIP1L1-PDGFRA
Myeloproliferative variant of the hypereosinophilic syndrome
One of three myeloproliferative and 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 associated with PDGFRA rearrangement that is associated with FIP1L1-PDGFRA formed as a result of a cryptic deletion at 4q12. Presentation is generally as chronic eosinophilic leukemia (CEL) but can be acute myeloid leukemia (AML) as well.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9960/3.) This is a multisystem disorder. The peripheral blood and bone marrow are always involved. Tissue infiltration by eosinophils can lead to tissue damage in the heart, lungs, central and peripheral nervous system, skin and GI tract. Spleen is enlarged in the majority of cases.
Definitive Diagnostic Methods
FIP1L1-PDGFRA fusion gene resulting from cryptic del (4) (q12)