Successful Heart Transplantation for Unreversible Endomyocardial Fibrosis Related to FIP1L1-PDGFRA Chronic Eosinophilic Leukemia

Fassnacht, F.; Roumier, Mathilde; Fouret, Pierre; Lévy, Vincent; Varnous, Shaïda; Russel, S.; Lefèvre, Guillaume; Kahn, Jean‐Emmanuel

doi:10.1097/tp.0000000000000939

Cited by 7 publications

(6 citation statements)

References 4 publications

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“…No IM‐treated patient showed features of accelerated or blast crisis disease. Lastly, two patients (including a previously reported case 29 ) underwent successful cardiac transplantation for refractory heart failure in the context of severe endomyocardial fibrosis ( Figure S3A).…”

Section: Resultsmentioning

confidence: 95%

Epidemiology, clinical picture and long‐term outcomes ofFIP1L1‐PDGFRA‐positive myeloid neoplasm with eosinophilia: Data from 151 patients

et al. 2020

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FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been identified. One hundred and fifty-one patients with F/P+ MN-eo (143 males; mean age at diagnosis 49 years; mean annual incidence: 0.18 case per million population) were included in this retrospective nationwide study involving all French laboratories who perform the search of F/P fusion gene (study period: 2003-2019). The main organs involved included the spleen (44%), skin (32%), lungs (30%), heart (19%) and central nervous system (9%). Serum vitamin

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Section: Resultsmentioning

confidence: 95%

Epidemiology, clinical picture and long‐term outcomes ofFIP1L1‐PDGFRA‐positive myeloid neoplasm with eosinophilia: Data from 151 patients

et al. 2020

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“…In case of persistent eosinophilia and subsequent occurrence of endomyocardial fibrosis, heart surgery with resection of fibrotic endocardium (endomyocardectomy) combined with valve repair or replacement can be considered ( 159 ). Finally, in case of refractory end-stage HF, orthotopic heart transplantation has been reported to be safe and feasible in both EGPA and HES ( 160 , 161 ).…”

Section: Eosinophilic Myocarditismentioning

confidence: 99%

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

et al. 2022

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The field of inflammatory disease of the heart or “cardio-immunology” is rapidly evolving due to the wider use of non-invasive diagnostic tools able to detect and monitor myocardial inflammation. In acute myocarditis, recent data on the use of immunomodulating therapies have been reported both in the setting of systemic autoimmune disorders and in the setting of isolated forms, especially in patients with specific histology (e.g., eosinophilic myocarditis) or with an arrhythmicburden. A role for immunosuppressive therapies has been also shown in severe cases of coronavirus disease 2019 (COVID-19), a condition that can be associated with cardiac injury and acute myocarditis. Furthermore, ongoing clinical trials are assessing the role of high dosage methylprednisolone in the context of acute myocarditis complicated by heart failure or fulminant presentation or the role of anakinra to treat patients with acute myocarditis excluding patients with hemodynamically unstable conditions. In addition, the explosion of immune-mediated therapies in oncology has introduced new pathophysiological entities, such as immune-checkpoint inhibitor-associated myocarditis and new basic research models to understand the interaction between the cardiac and immune systems. Here we provide a broad overview of evolving areas in cardio-immunology. We summarize the use of new imaging tools in combination with endomyocardial biopsy and laboratory parameters such as high sensitivity troponin to monitor the response to immunomodulating therapies based on recent evidence and clinical experience. Concerning pericarditis, the normal composition of pericardial fluid has been recently elucidated, allowing to assess the actual presence of inflammation; indeed, normal pericardial fluid is rich in nucleated cells, protein, albumin, LDH, at levels consistent with inflammatory exudates in other biological fluids. Importantly, recent findings showed how innate immunity plays a pivotal role in the pathogenesis of recurrent pericarditis with raised C-reactive protein, with inflammasome and IL-1 overproduction as drivers for systemic inflammatory response. In the era of tailored medicine, anti-IL-1 agents such as anakinra and rilonacept have been demonstrated highly effective in patients with recurrent pericarditis associated with an inflammatory phenotype.

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“…LE is often fatal with high in-hospital mortality, 1 and different mechanical circulation supports have been demonstrated to be useful. 2,3 To our knowledge, this is the first report in which the BiVAD was chosen as bridge to HTx. Moreover, our case also showed the useful role of the BiVAD in lowering the trend of peripheral hypereosinophilia, exploiting its ability to reduce the myocardial stress and the systemic inflammatory response.…”

Section: A Case Of Hypereosinophilic Syndrome With Loeffler Endocarditis Successfully Bridged To Heart Transplantation By Biventricular Mmentioning

confidence: 94%

A case of hypereosinophilic syndrome with Loeffler endocarditis successfully bridged to heart transplantation by biventricular mechanical support

2021

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Successful Heart Transplantation for Unreversible Endomyocardial Fibrosis Related to FIP1L1-PDGFRA Chronic Eosinophilic Leukemia

Cited by 7 publications

References 4 publications

Epidemiology, clinical picture and long‐term outcomes ofFIP1L1‐PDGFRA‐positive myeloid neoplasm with eosinophilia: Data from 151 patients

Epidemiology, clinical picture and long‐term outcomes ofFIP1L1‐PDGFRA‐positive myeloid neoplasm with eosinophilia: Data from 151 patients

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

A case of hypereosinophilic syndrome with Loeffler endocarditis successfully bridged to heart transplantation by biventricular mechanical support

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