Cardiac involvement in hypereosinophilic syndrome.
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ABSTRACT:
SUBMITTER: Sen T
PROVIDER: S-EPMC2801956 | biostudies-literature | 2009
REPOSITORIES: biostudies-literature
Publications
Cardiac involvement in hypereosinophilic syndrome.
Sen Taner T Gungor Omer O Akpinar Ibrahim I Cetin Mustafa M Tufekcioglu Omac O Golbasi Zehra Z
Texas Heart Institute journal 20090101 6
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Project description:A 9-year-old boy with hypereosinophilic syndrome (HES) was referred for cardiac magnetic resonance (CMR) imaging following an abnormal echocardiogram that showed a large mass layered on the inferolateral wall of the left ventricle, causing secondary severe mitral regurgitation. Cardiac involvement in HES usually affects the ventricular apex. In our case, CMR confirmed the presence of a large mural thrombus of 0.9 cm × 4.2 cm. This unusual cardiac involvement in HES was diagnosed in its intermediate thrombotic stage. CMR is very sensitive and specific in staging the disease. It explained the etiology of mitral regurgitation and guided therapy, especially when echocardiography was nonconclusive.
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Project description:BackgroundHypereosinophilic syndrome (HES) is a rare haematologic disorder defined by persistent eosinophilia associated with organ damage. Cardiac involvement occurs in HES patients frequently and represents major cause of their morbidity and mortality.Case summaryA 66-year-old female patient underwent comprehensive cancer screening due to significant weight reduction. Screening showed negative results except for echocardiography, which revealed a large right ventricular mass imitating malignant cardiac tumour. Patient thus underwent biopsy of the intracardial mass. The procedure was complicated with right ventricular perforation causing cardiac tamponade, successfully resolved by immediate pericardiocentesis. Subsequent echocardiography revealed an echodense mass also in left ventricular apex, resembling a thrombus. Laboratory findings with mildly elevated eosinophil count and presence of formations in both ventricular apexes arose suspicion of non-malignant disease origin, specifically eosinophilic endomyocarditis. The presumption was supported by medical history of bronchial asthma, pansinusitis, and hypereosinophilia. Cardiac magnetic resonance (CMR) confirmed signs of eosinophilic endomyocarditis of both ventricles, together with presence of several intracardiac thrombi. However, despite intensive treatment, patient died due to progressive heart failure 3 months later.DiscussionAs seen in our case, establishing diagnosis of eosinophilic endomyocarditis may represent a challenge in clinical practice, since it represents a rare disease with variable clinical manifestations. However, presence of formations in both ventricles together with peripheral eosinophilia should raise suspicion of this diagnosis. Our case also highlights the importance of CMR imaging in diagnostic process of eosinophilic endomyocarditis, since it represents a non-invasive diagnostic modality able to detect distinctive signs of eosinophilic endomyocarditis.
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Project description:Hypereosinophilic syndrome (HES) is defined by persistently elevated blood eosinophil levels and is associated with evidence of organ damage. Cardiovascular involvement in HES is most commonly associated with Loffler endocarditis (cardiac HES). Cardiac HES is typically characterized by progressive subendocardial fibrosis with overlying mural thrombus formation, leading to restrictive dysfunction of the left ventricle. The thrombus from cardiac HES could result in cardiogenic stroke; however, most of the stroke cases with HES were not associated with huge thromboembolism rather multiple infarcts in the watershed area. The major clinical features of 97 previously reported cases of stroke with HES are as follows: the median age was 52 years, of which 61 (63%) were men; the initial presenting symptoms were neurological (73%), followed by headache (16%), respiratory symptoms (9%), and visual symptoms (9%). Almost half of the cases were diagnosed with cardiac HES. The characteristics of cardiac findings were mural thrombi, endomyocardial fibrosis, and a restrictive pattern of heart failure. Cerebral findings revealed 78 cases (80%) were described as multiple infarctions and 55 cases (57 %) were involved with watershed areas, whereas 11 cases (11％) were described as embolic stroke for one proximal large-vessel occlusion. Regarding treatment, 71 (73%), 28 (29%), and 16 (16%) patients were treated with steroids, anticoagulants, and antiplatelets, respectively. The overall mortality and recovery rates were 11% and 89%, respectively. Physicians should know most cases of stroke with HES are characterized by multiple infarctions in the watershed area, and cardiac HES is not always associated with stroke.
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Project description:BackgroundHypereosinophilic syndrome is a group of diseases defined by marked eosinophilia in blood or tissue and eosinophil-related clinical manifestations. Benralizumab is a monoclonal antibody against interleukin-5 receptor α, which is expressed on human eosinophils.MethodsIn this randomized, double-blind, placebo-controlled, phase 2 trial, we administered a series of three monthly subcutaneous injections of either benralizumab (at a dose of 30 mg) or placebo in 20 symptomatic patients who had PDGFRA-negative hypereosinophilic syndrome and an absolute eosinophil count of at least 1000 cells per cubic millimeter; all the patients were receiving stable therapy (drugs or dietary changes) for this disease. This regimen was followed by an open-label phase, during which the patient's background therapy could be tapered as tolerated, and an extension phase. The primary end point of the randomized phase was a reduction of at least 50% in the absolute eosinophil count at week 12.ResultsDuring the randomized phase, the primary end point occurred in more patients in the benralizumab group than in the placebo group (9 of 10 patients [90%] vs. 3 of 10 patients [30%], P = 0.02). During the open-label phase, clinical and hematologic responses were observed in 17 of 19 patients (89%) and were sustained for 48 weeks in 14 of 19 patients (74%); in the latter group, in 9 of 14 patients (64%), background therapies could be tapered. Bone marrow and tissue eosinophilia were also suppressed with benralizumab therapy. The most common drug-related adverse events, headache and an elevated lactate dehydrogenase level, occurred in 32% of the patients after the first dose of benralizumab and resolved within 48 hours in all patients. Other adverse events occurred with similar frequency in the two groups. Of the many potential predictors of response that were examined, only clinical disease subtype appeared to be associated with the initial response or relapse.ConclusionsIn this small phase 2 trial, patients with PDGFRA-negative hypereosinophilic syndrome who received benralizumab for 12 weeks had lower absolute eosinophil counts than those who received placebo. During the open-label phase, clinical and hematologic responses were sustained for 48 weeks in 74% of the patients. Adverse events did not limit treatment. (Funded by the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov numbers, NCT00001406 and NCT02130882.).
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