Involvement In Hypereosinophilic Syndrome Research Articles

A SERIES OF GENETIC EVENTS: FIP1L1/PDGFR REARRANGEMENT LEADING TO EOSINOPHILIC MYOCARDITIS

A SERIES OF GENETIC EVENTS: FIP1L1/PDGFR REARRANGEMENT LEADING TO EOSINOPHILIC MYOCARDITIS

Loeffler endocarditis with intracardiac thrombus: case report and literature review

BackgroundLoeffler endocarditis is a relatively rare and potentially life-threatening heart disease. This study aimed to identify the characteristic features of Loeffler endocarditis with intracardiac thrombus on a background of hypereosinophilic syndrome (HES).Case presentationWe described a 57-year-old woman with Loeffler endocarditis and intracardiac thrombus initially presenting with neurological symptoms, who had an embolic stroke in the setting of HES. After cardiac magnetic resonance (CMR), corticosteroids and warfarin were administered to control eosinophilia and thrombi, respectively. During a 10-month follow-up, the patient performed relatively well, with no adverse events. We also systematically searched PubMed and Embase for cases of Loeffler endocarditis with intracardiac thrombus published until July 2021. A total of 32 studies were eligible and included in our analysis. Further, 36.4% of recruited patients developed thromboembolic complications, and the mortality rate was relatively high (27.3%). CMR was a powerful noninvasive modality in providing diagnostic and follow-up information in these patients. Steroids were administered in 81.8% of patients, achieving a rapid decrease in the eosinophil count. Also, 69.7% of patients were treated with anticoagulant therapy, and the thrombus was completely resolved in 42.4% of patients. Heart failure and patients not treated with anticoagulation were associated with poor outcomes.ConclusionsCardiac involvement in HES, especially Loeffler endocarditis with intracardiac thrombus, carries a pessimistic prognosis and significant mortality. Early steroids and anticoagulation therapy may be beneficial once a working diagnosis is established. Further studies are needed to provide evidence-based evidence for managing this uncommon manifestation of HES.

Literature reviews of stroke with hypereosinophilic syndrome.

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.

2499 Idiopathic Eosinophilic Syndrome With Hepatic Involvement

INTRODUCTION: Hepatic involvement in hypereosinophilic syndrome is rare. CASE DESCRIPTION/METHODS: A 66-year-old gentleman was referred for evaluation of persistent eosinophilia, cough, wheezing, and abnormal liver chemistries. No abdominal pain, fever, weight loss, and bowel habit changes. No traveling outside the U.S. On exam his vital signs were within normal range. Eyes, scleral icterus; Lungs; wheezing the bases; Abdomen, soft, nontender, nondistended, negative for, shifting dullness, no organomegaly, no distended veins on the abdominal wall; Extremities, no edema; Skin, no icterus no bruising. His initial laboratory findings were as follows, WBC 15.3 × 109/L, Hgb of 15.3 g/dL, platelets of 438 × 109/L, absolute neutrophil count of 6.7 × 109/L, absolute lymphocyte of 2.7 × 109/L, absolute eosinophil count of 4.5 × 109/L, alkaline phosphatase of 234 U/L, AST of 51 U/L, ALT of 94 U/L, total bilirubin of 0.4 mg/dL, albumin of 4.8 g/dL. With respect to the liver related laboratory findings: negative viral hepatitis panel, ANA of 1:160, total IgG of 1010 mg/dL, IgG1 of 659 mg/dL, IgG2 of 258 mg/dL, IgG3 of 12.9 mg/dL and IgG4 of 28 mg/dL; F-actin IgG antibody 23.3 units; negative anti-smooth muscle antibodies; soluble liver antibody <20 units; negative AMA, alpha-1 antitrypsin level of 167 mg/dL; and ceruloplasmin of 27 mg/dL. Comprehensive studies for stool ova and parasites was unremarkable. Peripheral blood smear showed eosinophilia. Bone marrow biopsy, with flow cytometric analysis revealing hypereosinophilia showing aberrant expression of CD 56. Abdominal MRI revealed a 5.8 cm hemangioma in the left lobe; no hepatic steatosis. Myeloproliferative neoplasms (MPN) and platelet-derived growth factor receptor α (PDGFRA A/B) workup was negative. Liver biopsy showed many eosinophils within portal areas with foci of sinusoidal involvement compatible with idiopathic eosinophilic syndrome; chronic hepatitis (grade 2/4), with portal and periportal fibrosis (stage II/IV); no steatosis. After starting prednisone 60 mg oral daily, tapered over 4 weeks, his cough, wheezing, hepatic enzymes' elevation and eosinophilia have resolved. DISCUSSION: Hypereosinophilic syndrome with hepatic involvement is rare, and should be in the differential diagnosis especially when hepatic enzymes' elevation is associated with eosinophilia.

Long-term Follow-up of Severe Eosinophilic Hepatitis: A Rare Presentation of Hypereosinophilic Syndrome.

Idiopathic hypereosinophilic syndrome (HES) is a rare, heterogeneous disorder characterized by a strikingly high eosinophil count (>1,500 cells/μL), over a long period of time (>6 months), with end organ damage. We present a 60-year-old patient with idiopathic HES with isolated liver involvement, a rare systemic disease and a rare solid organ involvement. The patient had a thorough investigational work up until HES was established, including liver biopsy. He needed intensive immunosuppressive treatment at first with steroids, then with azathioprine in conjunction with a low dose of steroids. After 16 years of follow-up, the patient showed no evidence of liver dysfunction. To the best of our knowledge, this is the longest follow-up for a patient with HES-associated chronic hepatitis. Our observation suggests that, with appropriate treatment, liver involvement in HES may be well controlled without deterioration to advanced liver failure.

CNS Involvement in Hypereosinophilic Syndrome

CNS Involvement in Hypereosinophilic Syndrome

INTRAVENOUS, BUT NOT ORAL, STEROID REQUIRED TO CONTROL EOSINOPHILIA IN HYPEREOSINOPHILIC SYNDROME

Introduction Hypereosinophilic syndrome (HES) is a condition resulting in peripheral eosinophilia (>1500 cells/μL) with end organ damage. Flares of this disease can be controlled with corticosteroids, although alternate agents are typically used for baseline control. Case Description A 59-year-old woman with HES with pulmonary involvement, presented to the emergency department with hemoptysis, dyspnea, and fever. The patient had been tapering her prednisone dose of 60mg daily and was also taking hydroxyurea 500mg BID. Upon presentation, her eosinophil count was 1670 cells/μL. CT revealed diffuse groundglass opacities throughout the lungs. Bronchoalveolar lavage (BAL) revealed 55% eosinophils. Methylprednisolone 80mg IV every 8 hours was administered for 4 days. The patient clinically improved and her eosinophil count dropped to 370 cells/μL. Alternate steroids were attempted with the goal of switching to an oral route for taper and discharge. Intramuscular triamcinolone, intravenous dexamethasone and oral prednisone were attempted. The patient failed all therapies with rising eosinophilia to peak of 6320 cells/μL. She was administered mepolizumab 300mg subcutaneously for long-term control of eosinophilia and to facilitate tapering of methylprednisolone. She was discharged with a peripherally inserted central catheter for a 4 week taper. Her eosinophil count has since remained zero while on mepolizumab. Discussion This case illustrates a scenario where outpatient therapy with intravenous methylprednisolone was needed to treat eosinophilic lung involvement in HES. Use of intravenous methylprednisolone therapy may be considered as bridge to steroid sparing therapies. Treatment with mepolizumab is very effective for treatment of HES and can spare patients of long-term corticosteroid therapy and associated side effects. Hypereosinophilic syndrome (HES) is a condition resulting in peripheral eosinophilia (>1500 cells/μL) with end organ damage. Flares of this disease can be controlled with corticosteroids, although alternate agents are typically used for baseline control. A 59-year-old woman with HES with pulmonary involvement, presented to the emergency department with hemoptysis, dyspnea, and fever. The patient had been tapering her prednisone dose of 60mg daily and was also taking hydroxyurea 500mg BID. Upon presentation, her eosinophil count was 1670 cells/μL. CT revealed diffuse groundglass opacities throughout the lungs. Bronchoalveolar lavage (BAL) revealed 55% eosinophils. Methylprednisolone 80mg IV every 8 hours was administered for 4 days. The patient clinically improved and her eosinophil count dropped to 370 cells/μL. Alternate steroids were attempted with the goal of switching to an oral route for taper and discharge. Intramuscular triamcinolone, intravenous dexamethasone and oral prednisone were attempted. The patient failed all therapies with rising eosinophilia to peak of 6320 cells/μL. She was administered mepolizumab 300mg subcutaneously for long-term control of eosinophilia and to facilitate tapering of methylprednisolone. She was discharged with a peripherally inserted central catheter for a 4 week taper. Her eosinophil count has since remained zero while on mepolizumab. This case illustrates a scenario where outpatient therapy with intravenous methylprednisolone was needed to treat eosinophilic lung involvement in HES. Use of intravenous methylprednisolone therapy may be considered as bridge to steroid sparing therapies. Treatment with mepolizumab is very effective for treatment of HES and can spare patients of long-term corticosteroid therapy and associated side effects.

The Underrecognized Cardiac Manifestation of Hypereosinophilic Syndrome

Hypereosinophilic syndrome (HES) is a rare systemic disease in which cardiac manifestations are the leading cause of morbidity and mortality. HES involvement of the heart can often clinically mimic acute coronary syndrome (ACS) with the patient undergoing repeat cardiac catheterizations when no conclusive diagnosis is arrived at, and no effective treatment is suggested to alleviate recurrent symptoms of chest discomfort. Here we present an elusive case of eosinophilic myocarditis (EM) and advocate that a detailed review of history as well as obtaining all medical records be done to avoid unnecessary and repeated invasive investigations in order to provide safe, cost effective, and efficient care for our patients.

Cardiac involvement in hypereosinophilic syndrome.

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.

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

Background: Renal complication in hypereosinophilic syndrome (HES) is rare, with literature scarcely reporting association of this syndrome with glomerular involvement. While the direct effect of eosinophilic infiltration in tissues has been linked to histological damage of the HES, other mechanisms may account for renal involvement too. Case Presentation: We present a case of a 17-year-old male patient, with progressive edema, contact reactive erythematous skin lesions, acute kidney injury, nephrotic syndrome and progressive eosinophilia. His bone marrow biopsy revealed moderate hyperplasia with severe eosinophilia and atypical lymphocytes. His renal biopsy revealed glomeruli, enlarged in volume with mesangial expansion and hypercellularity and segmental thickening of capillary loops. Likewise, some glomeruli showed peripheral hyalinosis with synechiae to Bowman’s capsule. Tubules showed cloudy swelling, mild tubular atrophy and hyaline cylinders. Interstitial area showed infiltrated lymphomononuclear cells, focal with no evidence of eosinophils. Blood vessels were unaltered. Immunofluorescence identified glomeruli with granular mesangial IgM deposition. After corticosteroid treatment, eosinophilia and creatinine values regress to normal range. Conclusions: While our case may suggest the coexistence of two unrelated diseases, further studies are required to assess the pathophysiology of glomerular involvement in HES. Given the possibility that mechanisms other than the direct effect of eosinophils are involved in certain patients

Hypereosinophilic syndrome: endomyocardial biopsy versus echocardiography to diagnose cardiac involvement

ABSTRACTObjective: To compare echocardiograms and endomyocardial biopsies to diagnose cardiac involvement in hypereosinophilic syndrome.Methods: We examined the agreement between echocardiography and endomyocardial biopsies to detect cardiac involvement in hypereosinophilic syndrome by reviewing cases identified as hypereosinophilia or hypereosinophilic syndrome in Mayo Clinic databases from January 1978 through June 2009. Single-organ cases of eosinophilia such as eosinophilic fasciitis and eosinophilic gastroenteritis were excluded. We recorded echocardiogram and endomyocardial biopsy results including biopsy staining for eosinophil granule major basic protein (if performed). Clinical and laboratory features documented included presenting symptom(s), maximum total eosinophil count, dose of prednisone (if any) and eosinophil count at the time of endomyocardial biopsy, cardiac enzymes, serum tryptase level, electrocardiogram result, the result of testing for the FIP1L1-PDGFRA fusion gene, complications associated with the biopsy procedures and available follow-up information.Results: From a total of 387 patients’ records screened 288 met the criteria for hypereosinophilic syndrome and of these 240 had echocardiograms. Among these patients there were 138 normal echocardiograms, 67 had echocardiograms without findings of hypereosinophilic syndrome but with one or more other abnormalities, and 35 had echocardiograms with findings consistent with hypereosinophilic syndrome. Twenty-five patients from this group of 35 patients had both echocardiogram and endomyocardial biopsy. In 15 patients there was agreement between both endomyocardial biopsy and echocardiography as to the presence (n = seven) or absence (n = eight) for findings of cardiac involvement. In 10 of 25 patients test results diverged: 3 patients with positive echocardiographic changes did not have confirmatory findings by endomyocardial biopsy and seven patients with positive biopsy findings had echocardiograms without findings of hypereosinophilic syndrome.Conclusions: Echocardiograms and endomyocardial biopsies agree for presence or absence of cardiac involvement 60% of the time. Endomyocardial biopsy detected cardiac involvement in 7 patients in whom the echocardiogram was negative for findings of hypereosinophilic syndrome.

Eosinophilic myocarditis and hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a heterogeneous group of hematological disorders characterized by a chronic, unexplained hypereosinophilia with tissue damage. Cardiac involvement occurs in ∼20% of patients with HES and represents a major turning point. Cardiac injuries related to eosinophilia are divided into three chronological phases: eosinophilic infiltration, thrombosis, and fibrosis. We report a case of a 33-year-old woman diagnosed with HES, with pulmonary and gastrointestinal involvement and eosinophilic myocarditis in cardiogenic shock. The evolution was favorable with dobutamine, anticoagulation, corticosteroids, and later, β-blockers and angiotensin-converting enzyme inhibitors. Cardiac involvement in HES is rare but carries a poor prognosis. Corticosteroids are considered by many to be the mainstay of treatment. Although new treatments have been suggested, only a few seem promising.

Very early stage left ventricular endocardial dysfunction of patients with hypereosinophilic syndrome.

Cardiac involvement in hypereosinophilic syndrome (HES) patients entails significant morbidity and mortality. Left ventricular (LV) endocardial damage is important for the development of cardiac involvement in HES patients. However very early stage LV endocardial damage, such as prior to the first stage of an acute necrotic stage, remains uncertain. We studied 32 HES patients, all with normal conventional echocardiographic findings. Global radial and circumferential strain (GRS and GCS) were determined for each peak global strain curve from the mid-LV short-axis view, and global longitudinal strain (GLS) was averaged each peak global strain curve from standard apical views by means of two-dimensional speckle-tracking method. Thirty-one age-, gender-, LV ejection fraction-matched normal subjects were studied for comparison. GRS and GRS were similar for HES patients and normal controls, but GLS for HES patients was significantly lower than that for normal controls (16.2 ± 3.3 % vs 19.3 ± 2.9 %, p < 0.001). Furthermore, receiver operating characteristic curve analysis identified GLS ≤17.0 % as the best predictor of LV endocardial dysfunction with a sensitivity of 66 %, specificity of 78 %, and area under the curve of 0.781 (p = 0.0001). In conclusions, LV endocardial dysfunction pre-existed even in HES patients without apparent cardiac involvement. GLS as assessed with the two-dimensional speckle-tracking method is a promising tool for the better management of very early stage of HES patients.

Cardiac involvement in hypereosinophilic syndrome

(2016). Cardiac involvement in hypereosinophilic syndrome. Acta Cardiologica: Vol. 71, No. 1, pp. 75-76.

0009: Cardiac involvement in hypereosinophilic syndrome: role of multimodality imaging

Cardiac disease occurs in more than 50% of patients with idiopathic hypereosinophilic syndrome; it is the major cause of morbidity and mortality in patients with this syndrome. The overall mortality is high if untreated. We aim to rapport recent experience in these cardiac diseases, especiall echographic features and the contribution of multimodalities imaging. This retrospective, descriptive and multicenter study reports the findings of patients in cardiac centers of Marseille, Montpellier and Rennes between September 2008 and March 2014. Echographic, MRI cardiac, coronarographic, PET CT, biology and histologic findings are reported. Seven patients have been reported. Echocardiography is the reference diagnostic method. Classical findings are progressive endomyocardial thickening, apical obliteration of one or both ventricles by echogenic material suggestive of fibrosis or thrombus formation, posterior mitral leaflet involvement and papillary dysfunction resulting in severe mitral regurgitation. These features are involved in embolic events or heart failure. MRI helps refine the diagnosis. The characteristic irreversible stage shows a circumferential thickened fibrotic sub endocardium with wall thrombi. The CT identifies non ischemic thrombus excluding coronary artery disease by coronary imaging. Coronary angiography may reveal fibrosis by the smoothed appearance of the ventricular walls and apical filling. PET CT shows general signs of myocarditis, such as myocyte necrosis, in the first stage of the disease. Symptomatic treatment can lead to cardiac surgery (mitral valve replacement, resection of endomyocardial fibrosis). Each imaging technique plays a role in each of the three stages of the disease and the typical features of myocarditis, thrombus or fibrosis must be identified. Only seven cases of this rare disease have been identified in three french cardiac centers since 2008; this too small sample does not allow us to draw statistical conclusions. Abstract 0009 - Figure: endomyocardial thickening and mitral involvement

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor

Hypereosinophilic Syndrome With Cardiac Involvement: Early Diagnosis by Cardiac Magnetic Resonance Imaging

Cardiac manifestation is the major cause of morbidity in patients with hypereosinophilic syndrome (HES) if left untreated. Endomyocardial biopsy is currently the gold standard for diagnosis, although typical findings using noninvasive methods such as cardiac magnetic resonance (CMR), may suggest the diagnosis. The present case provides an example of the beneficial use of cardiac magnetic resonance for early diagnosis and follow-up of patients with cardiac involvement in hypereosinophilic syndrome.

Unusual Cause of Acute Mitral Regurgitation: Idiopathic Hypereosinophilic Syndrome

Idiopathic hypereosinophilic syndrome (HES) is a rare multisystem condition characterized by dysregulated overproduction of eosinophils. Cardiac involvement in HES is characterized by necrosis from infiltration of eosinophils and thrombus formation and, in the late stage, by fibrosis and chronic valvular regurgitation. We report a very unusual presentation of idiopathic HES with acute mitral regurgitation due to papillary muscle rupture. The transesophageal echocardiogram was suggestive of a flail posterior leaflet and suspicious for endocarditis. Intraoperatively, papillary muscle rupture was seen and the patient underwent mitral valve replacement. The pathologic examination of the valve revealed eosinophilic infiltration of the papillary muscle. The patient was treated with steroids and responded well clinically.

Cardiac involvement in hypereosinophilic syndrome

Anselmino, Monica; Novara, Marco; Bellone, Alessandra; Minelli, Massimo Author Information

From an isolated right ventricular thrombus to the diagnosis of the hypereosinophilic syndrome

This case concerns a 58-year-old male who presented with abdominal pain, anorexia, nausea, and exertional dyspnea of one month's duration. On further evaluation peripheral eosinophilia was conspicuous and eosinophilic infiltration was found in the gastrointestinal system and bone marrow. Echocardiography showed an isolated right ventricular thrombus with tricuspid valve involvement. Cardiac magnetic resonance imaging helped to confirm the diagnosis of cardiac involvement in hypereosinophilic syndrome without requiring a cardiac biopsy.