25 results match your criteria: "National Reference Center for Hypereosinophilic Syndromes[Affiliation]"

Innate immune cells appear to have an important implication in the resolution and/or the aggravation of the COVID-19 pathogenesis after infection with SARS-CoV-2. To better appreciate the role of these cells during COVID-19, changes in blood eosinophil, the neutrophil and monocyte count, and levels of surface protein markers have been reported. However, analyses at several timepoints of multiple surface markers on granulocytes and monocytes over a period of one month after a SARS-CoV-2 infection are missing.

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Interactions between eosinophils and IL-5Rα-positive mast cells in nonadvanced systemic mastocytosis.

J Allergy Clin Immunol

December 2024

University of Paris, Institut Imagine, INSERM, Paris, France; French Reference Center for Mastocytosis (CEREMAST), Hôpital Necker-Enfants Malades, AP-HP, Paris, France; Department of Hematology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France. Electronic address:

Background: Bidirectional interactions between eosinophils and mast cells (MCs) have been reported in various allergic diseases. Bone marrow (BM) eosinophilia, and to a lesser extent blood eosinophilia, is common in systemic mastocytosis (SM), but its significance remains unknown.

Objective: We described blood and BM eosinophil characteristics in SM.

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Article Synopsis
  • - The study investigates the link between eosinophilia and ocular vascular issues, highlighting the procoagulant effects of eosinophils, which can lead to various cardiovascular problems and eye conditions.
  • - A retrospective, multicenter analysis of 57 patients revealed that the majority experienced eye issues as the first sign of eosinophil-related diseases, with specific conditions like central retinal artery occlusion being common among them.
  • - The findings indicate a poor visual prognosis for these patients, with only 10% achieving full recovery, suggesting the need for urgent treatment and further research on eosinophilia's impacts.
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Lessons From Prospective Longitudinal Follow-up of a French APECED Cohort.

J Clin Endocrinol Metab

February 2025

Department of Endocrinology, Diabetology and Metabolism, Huriez Hospital, Lille University Hospital, F-59000 Lille, France.

Article Synopsis
  • APECED syndrome is a rare genetic disorder caused by mutations in the AIRE gene, typically characterized by a triad of symptoms including hypoparathyroidism, adrenal failure, and chronic mucocutaneous candidiasis (CMC), along with other non-endocrine issues.
  • In a national study involving 25 patients from 23 families, researchers identified 11 different variants of the AIRE gene, including two previously unreported variants, and found that a majority of patients displayed multiple clinical manifestations.
  • The study revealed significant immunological disturbances, such as NK cell lymphopenia and altered B lymphocyte homeostasis, and highlighted a variety of non-endocrine symptoms that could potentially be life-threatening, emphasizing the need for
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We investigated using a custom NGS panel of 149 genes the mutational landscape of 64 consecutive adult patients with tyrosine kinase fusion-negative hypereosinophilia (HE)/hypereosinophilic syndrome (HES) harboring features suggestive of myeloid neoplasm. At least one mutation was reported in 50/64 (78%) patients (compared to 8/44 (18%) patients with idiopathic HE/HES/HE used as controls; p < .001).

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Eosinophilic ascites is a rare disorder, reported in both adult and pediatric patients, characterized by high eosinophil counts in the peritoneal fluid. Eosinophilic ascites appears as a manifestation of various diseases such as parasitic and fungal infections, malignancy, and hypereosinophilic syndrome. It also represents an uncommon manifestation of eosinophilic gastroenteritis, usually treated with corticosteroids.

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Mepolizumab for the Treatment of Eosinophilic Cystitis: Reply.

J Investig Allergol Clin Immunol

July 2024

National Reference Center for Hypereosinophilic Syndromes, CEREO, France; Université Paris-Saclay, Assistance Publique - Hôpitaux de Paris, Department of Internal Medicine, Ambroise Paré Hospital, Boulogne-Billancourt, France.

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Introduction: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear.

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Efficacy of Mepolizumab for the Treatment of Eosinophilic Cystitis: A Report of 2 Cases.

J Investig Allergol Clin Immunol

June 2024

National Reference Center for Hypereosinophilic Syndromes, CEREO, Université Paris-Saclay, Assistance Publique - Hôpitaux de Paris, Department of Internal Medicine, Ambroise Paré Hospital, Boulogne-Billancourt, France.

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Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients.

J Autoimmun

September 2023

Department of Internal Medicine, Hôpital Cochin, AP-HP.Centre, Université Paris Cité, Paris, France; University Paris-Cité, F-75006, Paris, France. Electronic address:

Article Synopsis
  • A retrospective study on polyarteritis nodosa (PAN) analyzed data from 196 patients referred to a French study group between 2005 and 2019, highlighting significant changes in the disease's landscape.
  • Main symptoms included constitutional, neurological, skin, and musculoskeletal issues, with 28% of cases being secondary PAN due to conditions like myelodysplastic syndrome and cancers; most patients (98.5%) were treated with glucocorticoids.
  • The study found high relapse rates associated with older age and specific clinical features, with mortality linked to factors like age, necrotic purpura, kidney injury, and secondary PAN, emphasizing the need for improved patient management.
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Eosinophilic-related clinical manifestations are protean and the underlying conditions underpinning eosinophilia are highly diverse. The etiological workup of unexplained eosinophilia/hypereosinophilia can be challenging, and can lead sometimes to extensive, inappropriate, costly and/or invasive investigations. To date, guidelines for the etiological workup and management of eosinophilia are mainly issued by hematologists, and thus mostly cover the scope of clonal hypereosinophilic syndromes (HES).

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JAK inhibition for CD3 CD4 lymphocytic-variant hypereosinophilic syndrome.

Clin Immunol

June 2023

National Reference Center for Hypereosinophilic Syndromes, CEREO, France; Université Paris-Saclay, Assistance Publique - Hôpitaux de Paris, Department of Internal Medicine, Ambroise Paré Hospital, F-92100 Boulogne-Billancourt, France.

Alternatives are urgently needed in patients with CD3 CD4 lymphocytic-variant hypereosinophilic syndrome (L-HES) requiring high-level steroids or who are unresponsive and/or intolerant to conventional alternative therapies. We report five L-HES patients (44-66 years) with cutaneous involvement (n = 5) and persistent eosinophilia (n = 3) despite conventional therapies, who successfully received JAK inhibitors (tofacitinib n = 1, ruxolitinib n = 4). JAKi led to complete clinical remission in the first 3 months in all (with prednisone withdrawal in four).

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Article Synopsis
  • Eosinophilia, a condition marked by high eosinophil levels in the blood, can be significant in ICU patients but has been understudied regarding its causes and implications.
  • In a study of 620 ICU patients over 6 years, two groups based on the timing of eosinophilia were identified: early (within 24 hours) and delayed (after 24 hours), with varying causes and symptoms.
  • The survival rates at 60 days post-admission were similar for both groups, and specific risk factors related to mortality were identified, highlighting the need for tailored management approaches based on the timing of eosinophilia onset.
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Targeting IL-5/5R for the treatment of idiopathic chronic eosinophilic pneumonia.

J Allergy Clin Immunol Pract

April 2023

National Reference Center for Hypereosinophilic Syndromes, Suresnes, France; Department of Internal Medicine, Foch Hospital, Suresnes, France. Electronic address:

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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.

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Background: Eosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse.

Methods: The aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE.

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Article Synopsis
  • Ischemic stroke can occur in patients with clonal hypereosinophilic syndrome caused by the FIP1L1-PDGFRA fusion, which is responsive to the drug imatinib.
  • A study analyzed 16 male patients with a history of ischemic stroke and eosinophilia, finding a high incidence of bilateral infarctions and a significant proportion of strokes with unclear causes.
  • Treatment with imatinib showed promising results, with a low recurrence rate of strokes over a median follow-up period of 4.5 years, suggesting that this syndrome should be considered in unexplained stroke cases.
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Eosinophils have widespread procoagulant effects. Eosinophilic cardiovascular toxicity mostly consists of endomyocardial damage or eosinophilic vasculitis, while reported cases of venous thrombosis (VT) are scarce. We aimed to report on the clinical features and treatment outcomes of patients with unexplained VT and eosinophilia, and to identify predictors of relapse.

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Article Synopsis
  • FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare condition with limited epidemiological data; a retrospective study analyzed 151 patients in France from 2003-2019.
  • Imatinib mesylate (IM) is very effective, with 98% of treated patients achieving complete hematologic and molecular responses; however, a significant percentage of patients relapsed after stopping IM.
  • Factors such as the timing of IM initiation and duration of treatment were identified as independent predictors of relapse, suggesting that early and prolonged treatment may help reduce the chances of relapse.
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