Development of Loffler's endocarditis in FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome despite continuous imatinib mesylate therapy and continuous complete remission

作者：Vaclavik Jan^*; Szotkowski Tomas; Hutyra Martin; Kozak Jiri; Lukl Jan; Taborsky Milos

来源：American Journal of Hematology, 2010, 85(4): 296-299.

DOI：10.1002/ajh.21635

We present a case of a 32-year-old male with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome (HES), whose complete clinical, hematological, and molecular remission was achieved and maintained on 100 mg of imatinib mesylate daily. The markedly increased thickness of the left ventricular (LV) apical myocardium at the time of the diagnosis decreased very quickly after imatinib and warfarin therapy and there was no detectable intramural thrombus on repeated echocardio-graphic check-ups. After 28 months of imatinib therapy and 16 months after warfarin withdrawal, both asymptomatic endocardial fibrosis and LV apical thrombus developed, consistent with the diagnosis of Loffler's endocarditis, and these findings were confirmed by cardiac magnetic resonance imaging (MRI). Resumption of warfarin therapy led to the disappearance of the apical thrombus after 7 months, whereas endomyocardial fibrosis persists and the LV systolic and diastolic functions remain normal. To our knowledge, no similar case of the development of Loffler's endocarditis during imatinib therapy with continuous complete remission of HES has been described up to the present. We suggest that frequent echocardiographic or preferably gadolinium-enhanced MRI check-ups are necessary and that long-term prophylactic oral anticoagulation should be considered in all patients with FIP1L1/PDGFRalpha-positive HES treated with imatinib.

• 出版日期2010-4