Hypereosinophilic Syndrome Research Articles

Acute Border Zone Infarcts in Hypereosinophilic Syndrome

Acute Border Zone Infarcts in Hypereosinophilic Syndrome

Management of multiple valve replacement operations for a young patient with hypereosinophilic syndrome and valvular heart disease: a case report.

Hypereosinophilic syndrome (HES) is characterized by the overproduction of eosinophils and manifests as valvular disease and thrombogenesis. Herein, we report our experience with a patient with HES requiring multiple reoperations for prosthetic heart valve replacement via median sternotomy. The patient was a 54-year-old man who had undergone four valve replacement operations via median sternotomy (three mitral valve replacements and one double valve replacement) because of valvular diseases complicated by HES since he was 26years old. All the artificial valves were bioprosthetic to prevent thrombotic events. At presentation, he had developed structural deterioration of the artificial aortic valve with severe stenosis. His prosthetic mitral valve did not fulfil the criteria for intervention, as it exhibited only mild regurgitation and no stenosis. The explanted mitral prosthetic valve at the previous (fourth) surgery had exhibited eosinophilic infiltration, resulting in the introduction of cyclosporin for poorly controlled HES. We conducted re-aortic valve replacement via a fifth median sternotomy using a bioprosthetic valve, and no eosinophilic infiltration was observed in the explanted valve. The patient was discharged on postoperative day 15 without complications. Controlling eosinophil count during the pre- and postoperative course is vital in treating patients with HES after valve replacement surgery. A holistic management and therapeutic strategy, including prosthetic valve selection and medication for HES is required to improve outcomes of patients with HES and heart valve disease.

Abstract 4127891: Value of Left Ventricular and Left Atrial Strains in Loeffler Endocarditis and Hypereosinophilic Syndrome

Background: Hypereosinophilic syndrome (HES) is a rare disease that causes organ damage and thrombosis due to sustained eosinophilia of the blood or tissues. Loeffler endocarditis (LE) is defined as cardiac involvement in the setting of HES. There are limited data on the diagnostic and prognostic impact of strain imaging on LE. Methods: A single-center retrospective cohort study was performed. Patients with eosinophilia between January 2000 and January 2023 were identified. We included patients who met the current diagnostic criteria for HES and underwent echocardiography prior to treatment. LE was defined as the presence of eosinophils in the myocardium by an endomyocardial biopsy, endocardial thickening, or left ventricular thrombus due to HES. We evaluated the composite events defined as CV death and stroke at diagnosis and during follow-up. Results: Of 1,664 patients with eosinophilia, 34 patients with HES were included (10 with LE and 24 without LE). In the entire cohort, the mean age was 57 ± 16 years and 41% were male. The median follow-up duration was 85 months (25th -75th percentile: 41-145 months). The composite events occurred in 6 patients. Left ventricular ejection fraction (LVEF) was similar in both groups (60% vs. 61%, p = 0.691). Patients with LE had significantly lower left ventricular global longitudinal strain (LV-GLS) (-9.7% vs. -15.5%, p = 0.001), left atrial (LA) reservoir strain (21.0% vs. 32.1%, p = 0.021) and LA conduit strain (-9.7% vs. -19.2%, p <0.001), compared to patients without LE. There were no significant differences in LA booster strain (-11.3% vs. -12.8%, p = 0.606) and right ventricular free wall strain (-17.5% vs. -23.4%, p = 0.083). All LE patients and half of non-LE patients had LV-GLS worse than -16 %. There was a significant difference in the compsoite events for patients with LV-GLS worse than -16% (28.6% vs. 0.0%, p = 0.049). Conclusions: LE was associated with significantly worse LV-GLS and left atrial strain compared to patients without LE. Worse LV-GLS was associated with a higher frequency of the composite events.

Exploring Cardiac Variability in Hypereosinophilia: Clinical Insights and Echocardiographic Findings

Introduction: The cardiac manifestations of hypereosinophilia include mainly endomyocardial fibrosis, which is the most frequently observed form. However, eosinophilic cardiopathy can manifest itself in different ways, affecting the various layers of the heart and presenting a variety of clinical and echocardiographic presentations. Aim: The aim of our study is to describe the various aspects of cardiac involvement observed in hypereosinophilia, based on our case series and data from the literature. Methods: This is a retrospective, descriptive study of five observations illustrating the diversity of eosinophilic cardiopathy, from the cardiology department of the Mohammed VI University Hospital in Marrakech, over a period of 12 months. Results: Over the period of the study, five patients presented with cardiac damage in the context of hypereosinophilia. The mean age was 53.8 years, with a predominance of women (sex ratio 0.66). In the majority of cases, the discovery of cardiac involvement was incidental to a cardiovascular check-up requested during the course of their medical condition. All cases had high levels of hypereosinophilia, with an average of 5681. These observations illustrate the wide variety of aetiologies of hypereosinophilia, all of which were represented: 3 cases of Churg-Strauss syndrome, 1 case of idiopathic hypereosinophilia syndrome, and 1 case of DRESS syndrome. The cardiac disorders observed were hypokinetic cardiomyopathy at the dilated stage in 60% of cases, acute eosinophilic myocarditis in 20%, and acute pericarditis in the remaining cases. The outcome was marked by an improvement in the FE in 60% of cases, stabilisation in 20%, and a fatal outcome in the remainder. Treatment is based on symptomatic therapy of the CHF and treatment of the underlying aetiology. Discussion and Conclusion: Eosinophils are cytotoxic through the release of granular proteins that initially attack the endocardium, leading to thrombosis and embolic events. Subsequently, this aggression contributes to fibrosis and valvular complications. Cardiac damage can also manifest itself as potentially serious acute myocarditis or pericarditis, which can progress to cardiac tamponade. These cardiac disorders and the diversity of possible aetiologies serve as a reminder that vital prognosis may be at stake in the acute phase, and to emphasise that functional prognosis depends on early echocardiographic screening and rapid initiation of treatment to limit the risk of thrombo-embolic and fibrosing complications.

Role of Eosinophils in Immunity, Allergy, and Inflammation: Friend or Foe

Eosinophils are multifunctional granulocytes involved in both protective and pathological immune responses. Traditionally recognized for their role in host defense against parasitic infections, eosinophils have gained significant attention for their involvement in allergic diseases, such as asthma, allergic rhinitis, and atopic dermatitis, where they are often considered detrimental. Eosinophils contribute to tissue damage and inflammation by releasing cytotoxic granule proteins, cytokines, and chemokines, perpetuating chronic inflammation. In conditions such as eosinophilic gastrointestinal disorders and hypereosinophilic syndrome, eosinophils infiltrate tissues and exacerbate inflammation, leading to organ dysfunction. However, eosinophils also exhibit beneficial roles, including modulating immune responses, promoting tissue repair, and maintaining tissue homeostasis. Recent research has highlighted their regulatory functions, where they interact with other immune cells to mediate both pro- and anti-inflammatory responses. The dual nature of eosinophils—both as protectors in immunity and contributors to pathology—presents a complex challenge in understanding their full impact on health and disease. Advances in biologic therapies targeting eosinophil activity, such as IL-5 inhibitors, offer promising treatment options for eosinophil-associated disorders. This review explores the diverse roles of eosinophils in immunity, allergy, and inflammation, examining both their protective and harmful functions, while discussing therapeutic strategies aimed at modulating eosinophil activity in various diseases. Keywords: Eosinophils, allergy, inflammation, immune regulation, IL-5 therapy

Dupilumab use in patients with hypereosinophilic syndromes: a multicenter case series and review of the literature

BackgroundHypereosinophilic syndromes (HES) are defined as hypereosinophilia with eosinophil-related clinical manifestations, some of which overlap in presentation with asthma, atopic dermatitis, eosinophilic esophagitis, and/or chronic rhinosinusitis with nasal polyps (CRSwNP). Dupilumab is approved to treat these conditions but can induce a transient rise in the absolute eosinophil count (AEC) and rare eosinophil-related complications. ObjectiveTo determine whether eosinophil-related complications of dupilumab are increased in HES. MethodsRetrospective chart review of patients with HES treated with dupilumab enrolled on an IRB-approved research protocol at the National Institutes of Health (NCT00001406) or receiving care at Walter Reed National Military Medical Center. Clinical response and treatment-emergent adverse events were recorded. Serum mediators were assessed in a subset of patients before and after dupilumab using stored samples. ResultsAmong the 28 patients (15 male, 13 female; median age 41.5), the most common prescribing indication for dupilumab was CRSwNP (n=11). Twenty-three patients (82%) showed significant clinical improvement on dupilumab. Hypereosinophilia (AEC >1.5x109/L) recurred or worsened in 9/20 patients on dupilumab monotherapy. Moreover, 4/20 (20%) patients developed an eosinophil-related complication with dupilumab discontinuation and/or addition of eosinophil-lowering therapy. None of the 8 patients who received dupilumab while in hematologic remission on an eosinophil-lowering biologic developed hypereosinophilia or an eosinophil-related complication. Serum IgE and eotaxin levels decreased on dupilumab therapy. ConclusionThese data suggest that dupilumab is effective in treating residual symptoms in HES patients but that the incidence of eosinophil-related complications is increased. Concomitant eosinophil-lowering therapy may reduce the risk of eosinophil-related complications during dupilumab therapy in patients with HES.

Clinical picture, outcomes and predictors of relapse in eosinophilia-associated coronary vasospasm: data from a European multicentric study

Abstract Background and aims Eosinophilia-associated coronary vasospasm is an under-recognized and scarcely reported life-threatening condition for whose management is undefined. Preliminary data suggest that eosinophil-targeted therapies could be beneficial. Our aim is to provide an in-depth report of the clinical picture, outcomes and predictors of relapse in patients with eosinophilia-associated coronary vasospasm. Methods European multicentric retrospective study, gathering twenty centers across France, Italy and Spain. Patients of 15 years or more with definite or suspected coronary vasospasm according to the 2017 International standardization of diagnostic criteria for vasospastic and concomitant (± 48 hours) eosinophilia (threshold: 0.5 x 109/L) were included in this study. Outcomes included vasospasm relapse and other major cardiovascular events (MACE) during follow-up. Predictors of relapse were assessed using bidirectional stepwise regression analysis. Results Thirty-seven patients (median age: 52 [42-64] years, 43% women) were included, among which 16 (43%) had aspirin-exacerbated respiratory disease, 16 (43%) and 13 (35%) fulfilled classification criteria for Eosinophilic Granulomatosis with Polyangiitis (all with negative antineutrophil cytoplasmic antibodies) and Hypereosinophilic syndrome, respectively. There was no predominant coronary territory involved. A iatrogenic trigger was evidenced in 7 (19%) patients, consisting in all cases but one of non-steroidal anti-inflammatory drugs. After a median follow-up of 32 [12-63] months, 20 (54%) patients relapsed (median time to first relapse: 10 [5−29] months), despite vasodilators regimen in 95% of cases. Besides coronary vasospasm(s), 13 (35%) patients experienced an additional MACE. In multivariate analysis, persistent eosinophilia (i.e. absolute eosinophil count > 0.5 x 109/L at the time of coronary vasospasm relapse or at last follow-up for relapsing and non-relapsing patients respectively) was the sole indepedendent predictor of relapse (Odds Ratio 8.87 [3.28-24.82]; p<0.001). All six patients treated with interleukin-5 biologics remained relapse-free under treatment. Conclusions Eosinophilia-associated coronary vasospasm can occur in patients with type 2 inflammation. Long-term normalization of absolute eosinophil counts appears crucial in preventing the recurrence of eosinophilia-associated coronary vasospasm, while conventional use of vasodilators seems ineficient.

A rare case of PDGFRA-positive chronic eosinophilic leukemia.

Chronic eosinophilic leukemia (CEL) is a clonal proliferation of eosinophilic precursors resulting in a persistently elevated number of eosinophils in blood, bone marrow, and peripheral tissues. The vaguely overlapping clinicopathological picture of CEL with idiopathic hypereosinophilic syndrome (IHES) often adds to the diagnostic dilemma. Here, we report a rare case of 49-year-old male who presented with multiorgan damage. Peripheral blood smear revealed predominantly eosinophils with dysplastic changes, shift to left in eosinophilic series, and 2% blasts. The bone marrow showed 5% blasts and many eosinophils, precursors of eosinophils, and myeloid and megakaryocytic proliferation. Till date, CEL is a very rare entity. Its presentation with cardiac and neurological manifestations is still rare.

Phase II trial of imatinib mesylate in patients with PDGFRA/B-negative hypereosinophilic syndrome.

The role of imatinib in PDGFRA/B-negative hypereosinophilic syndromes (HES) is controversial because of the heterogeneity of HES and the scarcity of prospective studies. We conducted a phase II clinical trial to evaluate the efficacy of imatinib in PDGFRA/B-negative HES. Thirty-two patients were treated with imatinib (100-400 mg daily), and the molecular basis of their response was identified using whole-exome sequencing (WES) and whole-transcriptome sequencing (WTS). The haematological response rate was 46.9%, with a complete haematological response (CHR) rate of 18.8%. The median time to response was 1.5 months. Among the six patients who achieved CHR, five maintained it until the 24th cycle of imatinib and one lost response after 20 months. The median progression-free survival was 4.3 months. WES and WTS were conducted for 11 patients. The number of non-silent mutations did not differ between responders and non-responders. Nine differentially expressed genes, including SNORD15A, were downregulated in responders. STAT5B::RARA, PAK2::PIGX, and FIP1L1::CHIC2 fusions were identified in patients with sustained responses, and RNF130::BRAF and WNK1::KDM5A fusions were identified in non-responders. Imatinib, along with an appropriate biomarker, could be a promising option for PDGFRA/B-negative HES.

A-170 Hypereosinophilic Syndrome and t(5;15): Case Report and Literature Review

Abstract Background Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterized by persistent eosinophilia (absolute eosinophil count >1,500/mm3), classified into primary/clonal, reactive and idiopathic. After ruling out secondary causes of eosinophilia (parasitic infections, allergic conditions, vasculitis and collagenosis, drugs, eosinophilic lung disease, allergic gastroenteritis and metabolic conditions such as adrenal insufficiency), the main hypotheses become oncohematological diseases. Main entities to consider are myeloid/lymphoid neoplasms associated with eosinophilia and rearrangement of PDGFRA, FDGFRB, FGFR1 or PCM1-JAK2 and chronic eosinophilic leukemia. Conventional and molecular cytogenetics are the basis for classification and diagnosis of various hematological malignancies. This report describes a rare case of HES with t(5;15) (q33;q22). Methods A 29-year-old male patient was admitted with leukocytosis with the following exams: hemoglobin 6.5g/dL, hematocrit 21.7%, platelets 71,000/mm3, leukocytes 56,190/mm3 (60% neutrophils with a left shift to pro-myelocyte; 6% monocytes and 24% eosinophils), DHL 277 U/L, B12 vitamin 932 pg/mL, folic acid 1.72ng/mL, ferritin 913ng/mL. Non-reactive results were detected for antinuclear antibody, rheumatoid Factor, HIV, hepatitis B and C, syphilis and chagas disease. Bone marrow was hypercellular for age with absolute granulocytic predominance (G:E ratio 64:1) and eosinophilia (38%) in all maturational stages without dysplasia. Bone marrow flow cytometry did not detect neither increases in blasts nor anomalous immunophenotype. Bone marrow biopsy demonstrated 99% cellularity with normal blasts. Cytogenetics with translocation between the long arms of chromosome 5 and 15 in 15 of 20 metaphases analyzed - 46,XY,t(5;15)(q33;q22). No other molecular tests were available for this patient. Results In the case described t(5;15)(q33;q22) was found, which may be related to the TP53BP1::PDGFRB gene rearrangement. This breakpoint generates a chimeric oncoprotein similar to other tyrosine kinase fusions responsive to Imatinib. 53BP1 is a cellular DNA damage response component that binds wild type (non-mutant) p53. Oncogenesis occurs since the coiled-coil domains of TP53BP1 can mediate the homodimerization of PDGFRB and the constitutive activation of its tyrosine kinase activity; on the other hand, the DNA damage response of TP53BP1 may be disrupted. Gene rearrangements involving PDGFRA and FDGFRB are associated with response to tyrosine kinase inhibitors. Elevated vitamin B12 is a common finding in myeloproliferations with eosinophilia due to the increased production of haptocorrins, which binds to vitamin B12 in serum and in other tissues. Conclusions To our knowledge, in the literature there is only one described case of Myeloproliferative Neoplasia with Eosinophilia related to t(5;15)(q33;q22).

More Than Just Stool: Hypereosinophilic Syndrome Presenting as Persistent Diarrhea in a Patient With Ulcerative Colitis

We highlight a case of non-infectious diarrhea that progressed despite supportive care, prompting further workup. A 50-year-old man presented with 1 week of voluminous diarrhea, nausea, emesis, and abdominal pain. His history included ulcerative colitis, primary sclerosing cholangitis, Clostridioides difficile treated via total colectomy with ileoanal pouch, treated disseminated Mycobacterium avium complex, and international travel. On physical exam he had normal vital signs, diffuse abdominal tenderness without peritoneal signs, and a rash. Lab work revealed leukocytosis with eosinophilia, hyponatremia, and elevated creatinine. Lactate, inflammatory markers, stool studies, and computed tomography of his abdomen were normal. Tissue biopsies of the duodenum, ileum, and rectal pouch collected during colonoscopy and push enteroscopy revealed prominent mucosal eosinophilia, confirming the diagnosis of hypereosinophilic syndrome (HES). Treatment with steroids led to significant improvement in all symptoms and blood eosinophilia. This case highlights the importance of aggressive workup of diarrhea with peripheral eosinophilia, especially in patients with ulcerative colitis: waiting 6 months to confirm the diagnosis via blood work would delay treatment. Notably, infection with Strongyloides stercoralis was ruled out in this case prior to treatment. This is an important step in the workup and management of HES to prevent progression of Strongyloidiasis.

Hypereosinophilic Syndrome with Skin Lesions and Secondary Membranous Nephropathy

Hypereosinophilic Syndrome with Skin Lesions and Secondary Membranous Nephropathy

Long-term remission in a patient with hypereosinophilic syndrome successfully treated with benralisumab

Long-term remission in a patient with hypereosinophilic syndrome successfully treated with benralisumab

Successful novel use of dupilumab for gastrointestinal involvement of idiopathic hypereosinophilic syndrome: case report and review of the literature.

Hypereosinophilic syndrome (HES) is characterized by blood and tissue hypereosinophilia leading to organ damage. Gastrointestinal involvement is the third most common manifestation. We present a patient with idiopathic HES with secondary eosinophilic esophagitis (EoE), gastritis, and enteritis, corticosteroids-dependent, azathioprine- and mepolizumab-refractory. The patient achieved clinical and histopathologic remission following dupilumab treatment. A 28year-old female presented with chronic episodic nausea and emesis since childhood and initial diagnosis of primary eosinophilic gastrointestinal disease (EGID), improved with corticosteroids, refractory to azathioprine. She was found to have peripheral eosinophilia and multifactorial anemia, with iron, B12, and folate deficiencies. Esophageal, gastric, duodenal, and terminal ileum biopsies showed significant eosinophilic infiltrate. Bone marrow biopsy at age 31 confirmed HES diagnosis. By age 32, she became total parental nutrition (TPN)-dependent. She failed trials of benralizumab and mepolizumab [anti-interleukin (IL)-5 inhibitors], and cromolyn (mast-cell stabilizer). After developing new esophageal stricture, we initiated dupilumab (IL-4/13 inhibitor), recently FDA-approved for EoE. After 9weeks, esophageal stricture, gut tissue eosinophilia, and prior intestinal ulcerations resolved. She ceased TPN and is tolerating a non-restricted diet, with complete symptom resolution. Our patient's complete remission with dupilumab shows promise for broadening its use in treating GI involvement in HES, along with primary EGIDs.

Acute Liver Injury and Bilateral Pulmonary Artery Thrombosis Due to Hypereosinophilic Syndrome.

A 46-year-old Japanese man was referred to our hospital because of a marked increase in his eosinophil count (22,870/μL) and elevated liver enzyme levels. Computed tomography (CT) showed thrombi measuring approximately 8 cm in both femoral veins. A liver biopsy revealed eosinophilic infiltration, hepatocyte necrosis, fibrosis, and multiple thrombi. We suspected acute liver injury and deep vein thrombosis associated with hypereosinophilic syndrome and initiated steroids and heparin treatment. Four days after starting treatment, the patient experienced sudden chest pain and cardiopulmonary arrest. CT revealed bilateral pulmonary artery thrombosis, and despite administration of a tissue plasminogen activator, the patient died.

Fulminant eosinophilic myocarditis and refractory ventricular arrhythmias requiring mechanical circulatory support: a case report

Abstract Background Fulminant eosinophilic myocarditis (EM) is a rare and often fatal condition that may present atypically and be complicated by ventricular arrhythmias. Treatment involves high-dose corticosteroids to suppress eosinophilia, as well as increasing use of mepolizumab, an anti-interleukin-5 antibody with evidence for long-term efficacy and safety. Case summary A 38-year-old woman presented to the emergency department with neck pain and fatigue, and after extensive investigation was diagnosed with EM secondary to idiopathic hypereosinophilic syndrome. The patient was treated with methylprednisolone for eosinophil suppression and warfarin due to the presence of biventricular apical thrombi. Despite previously stable haemodynamics, the patient had a cardiac arrest due to ventricular fibrillation on Day 11 of hospitalization, requiring 30 min of cardiopulmonary resuscitation and commencement of veno-arterial extracorporeal membrane oxygenation support due to refractory ventricular arrhythmias. The patient was urgently listed for heart transplant but a suitable match was not identified, and she was weaned to pharmacologic support on Day 34. The patient survived with minimal sequelae and has returned to full-time work. She remains on mepolizumab as a steroid-sparing agent, therapeutic anti-coagulation, and heart failure therapy. Discussion This report describes an atypical presentation of fulminant EM requiring weeks of mechanical circulatory support due to refractory arrhythmia rather than heart failure. The case also highlighted the benefits of non-invasive cardiac magnetic resonance imaging in both diagnosis and prognostication of EM, as well as the need to maintain a high index of suspicion for this rare disease.

A Challenging Case of Idiopathic Hypereosinophilic Syndrome: An Itching in the Brain

A Challenging Case of Idiopathic Hypereosinophilic Syndrome: An Itching in the Brain

Catastrophic Thrombosis: A Narrative Review.

Catastrophic thrombosis is a severe condition characterized by a hypercoagulable tendency, leading to multiple thromboembolic events in different blood vessels, usually within a short timeframe. Several conditions have been associated with the development of catastrophic thrombosis, including the catastrophic antiphospholipid syndrome, thrombotic anti-platelet factor 4 immune disorders, thrombotic microangiopathies, cancers, the hyper-eosinophilic syndrome, pregnancy, infections, trauma, and drugs. Thrombotic storm represents a medical emergency whose management represents a serious challenge for physicians. Besides the prompt start of anticoagulation, a patient's prognosis depends on early recognition and possible treatment of the underlying condition. In this narrative review, we summarize the main characteristics of catastrophic thrombosis, analyzing the various conditions triggering such life-threatening complication. Finally, an algorithm with the diagnostic workup and the initial management of patients with catastrophic thrombosis is presented.

Endomyocardial involvement in asymptomatic Latin American migrants with eosinophilia related to helminth infection: A pilot study.

Hypereosinophilic syndrome can produce cardiac involvement and endomyocardial fibrosis, which have a poor prognosis. However, there is limited information regarding cardiac involvement among migrants from Latin America with eosinophilia related to helminthiasis. We conducted a pilot observational study where an echocardiography was performed on migrants from Latin America with both eosinophilia (>450 cells/μL) and a diagnosis of helminth infection, and on migrants from Latin America without eosinophilia or helminth infection. Microbiological techniques included a stool microscopic examination using the Ritchie's formalin-ether technique, and a specific serology to detect Strongyloides stercoralis antibodies. 37 participants were included, 20 with eosinophilia and 17 without eosinophilia. Twenty (54.1%) were men with a mean age of 41.3 (SD 14.3) years. Helminthic infections diagnosed in the group with eosinophilia were: 17 cases of S. stercoralis infection, 1 case of hookworm infection, and 2 cases of S. stercoralis and hookworm coinfection. Among participants with eosinophilia, echocardiographic findings revealed a greater right ventricle thickness (p = 0.001) and left atrial area and volume index (p = 0.003 and p = 0.004, respectively), while showing a lower left atrial strain (p = 0.006) and E-wave deceleration time (p = 0.008). An increase was shown in both posterior and anterior mitral leaflet thickness (p = 0.0014 and p = 0.004, respectively) when compared with participants without eosinophilia. Migrants from Latin America with eosinophilia related to helminthic infections might present incipient echocardiographic alterations suggestive of early diastolic dysfunction, that could be related to eosinophilia-induced changes in the endomyocardium.

Managing Patients with Hypereosinophilic Syndrome: A Statement from the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC).

Hypereosinophilic syndrome (HES) encompasses a heterogeneous and complex group of different subtypes within the wider group of hypereosinophilic disorders. Despite increasing research interest, several unmet needs in terms of disease identification, pathobiology, phenotyping, and personalized treatment remain to be addressed. Also, the prospective burden of non-malignant HES and, more in general, HE disorders is currently unknown. On a practical note, shortening the diagnostic delay and the time to an appropriate treatment approach probably represents the most urgent issue, even in light of the great impact of HES on the quality of life of affected patients. The present document represents the first action that the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC) has finalized within a wider project aiming to establish a collaborative national network on HES (InHES-Italian Network on HES) for patients and physicians. The first step of the project could not but focus on defining a common language as well as sharing with all of the medical community an update on the most recent advances in the field. In fact, the existing literature has been carefully reviewed in order to critically integrate the different views on the topic and derive practical recommendations on disease identification and treatment approaches.