ArticleMedicine2014
The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype.
Article in Medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 2 of them syntheses that pooled it.
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Who cites it
43 citing papers in PubMed, 2 syntheses or guidelines pooled it, 122 citations in OpenAlex.
- French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes.Orphanet journal of rare diseases · 2023Guideline
- Longitudinal Evaluation of Segmental Arterial Mediolysis in Splanchnic Arteries: Case Series and Systematic Review.PloS one · 2016Pooled it
- Lymphocyte variant hypereosinophilic syndrome with severe eosinophilic pneumonia treated with mepolizumab: a case report.Central-European journal of immunology · 2026Article
- Expansion of multiple CD4The Journal of allergy and clinical immunology · 2025Article
- Hypereosinophilia: clinical and therapeutic approach in 2025.Current opinion in allergy and clinical immunology · 2025Review
- Exploring the boundaries between neoplastic and reactive lymphoproliferations: lymphoid neoplasms with indolent behavior and clonal lymphoproliferations-a report of the 2024 EA4HP/SH lymphoma workshop.Virchows Archiv : an international journal of pathology · 2025Review
- [Efficacy and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2025Article
- Hypereosinophilia in Children.Current allergy and asthma reports · 2025Review
- Lymphocytic Variant Hypereosinophilic Syndrome: Case Series From a Tertiary Referral Center in Canada.EJHaem · 2025Article
- T-cell lymphoproliferative disorder with a STAT3 mutation causing a lymphocytic variant of hypereosinophilic-like syndrome without eosinophilia.Journal of hematopathology · 2025Article
- Steroid-sparing benefits of biologic use in hypereosinophilic syndrome and substantial disease burden across subtypes.Frontiers in allergy · 2025Article
- Deciphering the Etiologies of Adult Erythroderma: An Updated Guide to Presentations, Diagnostic Tools, Pathophysiologies, and Treatments.American journal of clinical dermatology · 2024Review
- Cardiac Abnormalities in Hypereosinophilic Syndromes.Arquivos brasileiros de cardiologia · 2024Review
- Review
- Clinical and Therapeutic Intervention of Hypereosinophilia in the Era of Molecular Diagnosis.Cancers · 2024Review
- DECTIN-1: A modifier protein in CTLA-4 haploinsufficiency.Science advances · 2023Article
- Epidemiology, clinical presentation, and outcomes of 620 patients with eosinophilia in the intensive care unit.Intensive care medicine · 2023Article
- Case report: Serious unexpected vascular events in two patients with lymphocytic variant hypereosinophilic syndrome.Frontiers in cardiovascular medicine · 2023Article
- Approach to the patient with suspected hypereosinophilic syndrome.Hematology. American Society of Hematology. Education Program · 2022Review
- Cutaneous manifestations of lymphoid-variant hypereosinophilic syndrome.The British journal of dermatology · 2022Article
Corrections and comments
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Authors and funding
29 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The CD3-CD4+ aberrant T-cell phenotype is the most described in the lymphoid variant of hypereosinophilic syndrome (L-HES), a rare form of HES. Only a few cases have been reported, and data for these patients are scarce. To describe characteristics and outcome of CD3-CD4+ L-HES patients, we conducted a national multicentric retrospective study in the French Eosinophil Network. All patients who met the recent criteria of hypereosinophilia (HE) or HES and who had a persistent CD3-CD4+ T-cell subset on blood T-cell phenotyping were included. Clinical and laboratory data were retrospectively collected by chart review. CD3-CD4+ L-HES was diagnosed in 21 patients (13 females, median age 42 years [range, 5-75 yr]). Half (48%) had a history of atopic manifestations. Clinical manifestations were dermatologic (81%), superficial adenopathy (62%), rheumatologic (29%), gastrointestinal (24%), pulmonary (19%), neurologic (10%), and cardiovascular (5%). The median absolute CD3-CD4+ T-cell count was 0.35 G/L (range, 0.01-28.3), with a clonal TCRγδ rearrangement in 76% of patients. The mean follow-up duration after HES diagnosis was 6.9 ± 5.1 years. All patients treated with oral corticosteroids (CS) (n = 18) obtained remission, but 16 required CS-sparing treatments. One patient had a T-cell lymphoma 8 years after diagnosis, and 3 deaths occurred during follow-up.In conclusion, clinical manifestations related to CD3-CD4+ T cell-associated L-HES are not limited to skin, and can involve all tissue or organs affected in other types of HE. Contrary to FIP1L1-PDGFRA chronic eosinophilic leukemia patients, CS are always effective in these patients, but CS-sparing treatments are frequently needed. The occurrence of T-cell lymphoma, although rare in our cohort, remains a major concern during follow-up.
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