Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.7.10.1 (ERK)
 95,504 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Endomyocardial fibrosis (Loeffler's endocarditis) is the main cause of poor outcome in Hyper Eosinophilic Syndrome (HES) and Eosinophilic Leukemia (EL). Reversion of the cardiac damage has been seldom reported, and thrombi can superimpose on infiltrated walls, originating oembolic complications. The tyrosine kynase inhibitor imatinib has been recently employed in patients affected by HES or EL, with impressive results. We have treated with imatinib a young patient affected by Loeffler's endocarditis during EL. Loeffler's endocarditis was studied by transthoracic Doppler echocardiography with and without the contrast agent SonoVue. Cytogenetics, FISH and molecular analysis showed the presence of the FIP1L1/PDGFRA fusion gene, recently detected in a majority of HES patients. Standard echocardiography revealed a large infiltration of the apical region, with apparently pedunculate corpora floating in the LV chamber; after SonoVue injection, a thick endo-myocardial infiltration involving papillary muscles and tendinous chords appeared, which simulated mobile thrombi at standard echography. Treatment with low dose imatinib caused rapid regression of both eosinophilic proliferation and endomyocardiopathy. The fusion gene FIP1L1-PDGFRA was found significantly decreased after a few months of treatment. Using a contrast echocardiographic approach, we demonstrated the non-thrombotic origin of the "in plus" image in our patient and its rapid resolution following imatinib treatment. Imatinib is an excellent candidate for first line treatment of Loeffler's endocarditis, especially when the FIP1L1/PDGFA fusion gene is detected.
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PMID:Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. 1562 68

Loeffler's endocarditis is a clinical condition characterized by combination of three key findings: unexplained prolonged and marked eosinophilia (>1500 eosinophils/mm(3) ), absence of a primary cause of hypereosinophilia, and evidence of eosinophil-mediated organ damage. We report a case of a 55-year-old African American male with symptoms of heart failure. Hematology showed white blood cell count of 17 670/mm(3) with 63% eosinophils and an absolute eosinophil count of 11 133/mm(3) . Echocardiogram and computed tomography showed near complete obliteration of right ventricular cavity. Endomyocardial biopsy showed diffuse myocyte necrosis with extensive eosinophilic infiltration without fibrosis consistent with early Loeffler's endocarditis. Molecular and cytogenetic analyses of bone marrow cells were negative for FIP1L1-PDGFRA fusion, PDGFRB mutation, abnormal myeloid maturation, or a lymphoproliferative disorder. Flow cytometry showed no clonality excluding chronic eosinophilic leukemia. There was a complete resolution of symptoms and eosinophilia after 1 month of steroid therapy.
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PMID:Early-Stage Loeffler's Endocarditis with Isolated Right Ventricular Involvement: Management, Long-Term Follow-Up, and Review of Literature. 2717 73