Research & Literature

Idiopathic Hypereosinophilic Syndrome With Cutaneous Manifestations and Flame Figures: A Spectrum of Eosinophilic Dermatoses Whose Features Overlap With Wells' Syndrome.

Smith SM, Kiracofe EA, Clark LN, Gru AA

The American Journal of dermatopathology 2015; (37(12)):910-4 doi:10.1097/DAD.0000000000000279.

Hypereosinophilic syndrome: cardiac diagnosis and management.

Mankad R, Bonnichsen C, Mankad S

Heart (British Cardiac Society) 2016; (102(2)):100-6 doi:10.1136/heartjnl-2015-307959.

Severe Prolonged Cough as Presenting Manifestation of FIP1L1-PDGFRA+ Chronic Eosinophilic Leukaemia: A Widely Ignored Association.

Roufosse F, Heimann P, Lambert F, et al.

Respiration; international review of thoracic diseases 2016; (91(5)):374-9 doi:10.1159/000446076.

Targeted next-generation sequencing identifies a subset of idiopathic hypereosinophilic syndrome with features similar to chronic eosinophilic leukemia, not otherwise specified.

Wang SA, Tam W, Tsai AG, et al.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 2016; (29(8)):854-64 doi:10.1038/modpathol.2016.75.

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

Yamamoto T, Tanaka H, Kurimoto C, et al.

The international journal of cardiovascular imaging 2016; (32(9)):1357-1361 doi:10.1007/s10554-016-0917-x.

Idiopathic hypereosinophilic syndrome presenting with hepatitis and achalasia.

Cheung AC, Hachem CY, Lai J

Clinical journal of gastroenterology 2016; (9(4)):238-42 doi:10.1007/s12328-016-0661-8.

IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: A comparative case series.

Carruthers MN, Park S, Slack GW, et al.

European journal of haematology 2017; (98(4)):378-387 doi:10.1111/ejh.12842.

Eosinophil-derived IL-4 drives progression of myocarditis to inflammatory dilated cardiomyopathy.

Diny NL, Baldeviano GC, Talor MV, et al.

The Journal of experimental medicine 2017; (214(4)):943-957 doi:10.1084/jem.20161702.

Adhesion-induced eosinophil cytolysis requires the receptor-interacting protein kinase 3 (RIPK3)-mixed lineage kinase-like (MLKL) signaling pathway, which is counterregulated by autophagy.

Radonjic-Hoesli S, Wang X, de Graauw E, et al.

The Journal of allergy and clinical immunology 2017; (140(6)):1632-1642 doi:10.1016/j.jaci.2017.01.044.

Bone marrow morphology is a strong discriminator between chronic eosinophilic leukemia, not otherwise specified and reactive idiopathic hypereosinophilic syndrome.

Wang SA, Hasserjian RP, Tam W, et al.

Haematologica 2017; (102(8)):1352-1360 doi:10.3324/haematol.2017.165340.

Eosinophilic myocarditis and hypereosinophilic syndrome.

Boussir H, Ghalem A, Ismaili N, El Ouafi N

Journal of the Saudi Heart Association 2017; (29(3)):211-213 doi:10.1016/j.jsha.2016.11.001.

Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis.

Alam A, Thampi S, Saba SG, Jermyn R

Clinical medicine insights. Case reports 2017; (10()):1179547617723643 doi:10.1177/1179547617723643.

World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management.

Gotlib J

American journal of hematology 2017; (92(11)):1243-1259 doi:10.1002/ajh.24880.

[Pediatric idiopathic hypereosinophilic syndrome with pulmonary embolism: a case report and review of literature].

Dong N, Dong XY

Zhonghua er ke za zhi = Chinese journal of pediatrics 2017; (55(10)):775-779 doi:10.3760/cma.j.issn.0578-1310.2017.10.013.

Biologic Agents for the Treatment of Hypereosinophilic Syndromes.

Kuang FL, Klion AD

The journal of allergy and clinical immunology. In practice 2017; (5(6)):1502-1509 doi:10.1016/j.jaip.2017.08.001.

Eosinophilic Myocarditis.

Cheung CC, Constantine M, Ahmadi A, et al.

The American journal of the medical sciences 2017; (354(5)):486-492 doi:10.1016/j.amjms.2017.04.002.

Living with a rare disorder: a systematic review of the qualitative literature.

von der Lippe C, Diesen PS, Feragen KB

Molecular genetics & genomic medicine 2017; (5(6)):758-773 doi:10.1002/mgg3.315.

Hypereosinophilic syndrome in the differential diagnosis of pulmonary infiltrates with eosinophilia.

Zimmermann N, Wikenheiser-Brokamp KA

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2018; (121(2)):179-185 doi:10.1016/j.anai.2018.05.014.

A multimodality work-up of patients with Hypereosinophilia.

Hu Z, Boddu PC, Loghavi S, et al.

American journal of hematology 2018; (93(11)):1337-1346 doi:10.1002/ajh.25247.

Consultation for Elevated Blood Eosinophils: Clinical Presentations, High Value Diagnostic Tests, and Treatment Options.

Khoury P, Bochner BS

The journal of allergy and clinical immunology. In practice 2018; (6(5)):1446-1453 doi:10.1016/j.jaip.2018.04.030.

Benralizumab for PDGFRA-Negative Hypereosinophilic Syndrome.

Kuang FL, Legrand F, Makiya M, et al.

The New England journal of medicine 2019; (380(14)):1336-1346 doi:10.1056/NEJMoa1812185.

Pemphigus-like hypereosinophilic syndrome with FIP1L1-PDGFRA fusion gene: A challenging and uncommon clinical presentation.

Curto-Barredo L, Segura S, Ishii N, et al.

The Journal of dermatology 2019; (46(6)):531-534 doi:10.1111/1346-8138.14888.

Prevalence and spectrum of T-cell lymphoproliferative disorders in patients with Hypereosinophilia: A reference laboratory experience.

Shi M, Rech KL, Otteson GE, et al.

Annals of diagnostic pathology 2020; (44()):151412 doi:10.1016/j.anndiagpath.2019.151412.

Multiple Biological Aspects of Eosinophils in Host Defense, Eosinophil-Associated Diseases, Immunoregulation, and Homeostasis: Is Their Role Beneficial, Detrimental, Regulator, or Bystander?

Kanda A, Yasutaka Y, Van Bui D, et al.

Biological & pharmaceutical bulletin 2020; (43(1)):20-30 doi:10.1248/bpb.b19-00892.

Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics.

Kuang FL, Curtin BF, Alao H, et al.

The journal of allergy and clinical immunology. In practice 2020; (8(8)):2718-2726.e2 doi:10.1016/j.jaip.2020.04.025.

Causes of hypereosinophilia in 100 consecutive patients.

Moller D, Tan J, Gauiran DTV, et al.

European journal of haematology 2020; (105(3)):292-301 doi:10.1111/ejh.13437.

Eosinophilia Associated With CD3-CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients.

Carpentier C, Verbanck S, Schandené L, et al.

Frontiers in immunology 2020; (11()):1765 doi:10.3389/fimmu.2020.01765.

Efficacy and safety of mepolizumab in hypereosinophilic syndrome: A phase III, randomized, placebo-controlled trial.

Roufosse F, Kahn JE, Rothenberg ME, et al.

The Journal of allergy and clinical immunology 2020; (146(6)):1397-1405 doi:10.1016/j.jaci.2020.08.037.

Acute progressive stroke with middle cerebral artery occlusion caused by idiopathic hypereosinophilic syndrome: a case report.

Li QF, Zhang Q, Huang YF, Zhang ZX

BMC neurology 2020; (20(1)):361 doi:10.1186/s12883-020-01941-8.

Loeffler's Endocarditis: An Integrated Multimodality Approach.

Polito MV, Hagendorff A, Citro R, et al.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2020; (33(12)):1427-1441 doi:10.1016/j.echo.2020.09.002.

The multiple functions and subpopulations of eosinophils in tissues under steady-state and pathological conditions.

Kanda A, Yun Y, Bui DV, et al.

Allergology international : official journal of the Japanese Society of Allergology 2021; (70(1)):9-18 doi:10.1016/j.alit.2020.11.001.

Eosinophilia/Hypereosinophilia in the Setting of Reactive and Idiopathic Causes, Well-Defined Myeloid or Lymphoid Leukemias, or Germline Disorders.

Kelemen K, Saft L, Craig FE, et al.

American journal of clinical pathology 2021; (155(2)):179-210 doi:10.1093/ajcp/aqaa244.

A case of hypereosinophilic syndrome with STAT5b N642H mutation.

Ding F, Wu C, Li Y, et al.

Oxford medical case reports 2021; (2021(1)):omaa129 doi:10.1093/omcr/omaa129.

CD3-CD4+ Lymphocytic Variant Hypereosinophilic Syndrome: Diagnostic Tools Revisited.

Carpentier C, Schandené L, Dewispelaere L, et al.

The journal of allergy and clinical immunology. In practice 2021; (9(6)):2426-2439.e7 doi:10.1016/j.jaip.2021.01.030.

Idiopathic hypereosinophilic syndrome in hemodialysis patients: Case reports.

Mutsuyoshi Y, Hirai K, Morino J, et al.

Medicine 2021; (100(10)):e25164 doi:10.1097/MD.0000000000025164.

The increasing incidence and prevalence of hypereosinophilic syndrome in the United Kingdom.

Requena G, Logie J, Gibbons DC, et al.

Immunity, inflammation and disease 2021; (9(4)):1447-1451 doi:10.1002/iid3.495.

FIP1L1-PDGFRA-Associated Hypereosinophilic Syndrome as a Treatable Cause of Watershed Infarction.

Tennenbaum J, Groh M, Venditti L, et al.

Stroke 2021; (52(10)):e605-e609 doi:10.1161/STROKEAHA.121.034191.

Rare Diseases of the Oral Cavity, Neck, and Pharynx.

Reichel CA

Laryngo- rhino- otologie 2021; (100(S 01)):S1-S24 doi:10.1055/a-1331-2851.

Safety and Efficacy of Mepolizumab in Hypereosinophilic Syndrome: An Open-Label Extension Study.

Gleich GJ, Roufosse F, Chupp G, et al.

The journal of allergy and clinical immunology. In practice 2021; (9(12)):4431-4440.e1 doi:10.1016/j.jaip.2021.07.050.

Literature reviews of stroke with hypereosinophilic syndrome.

Ono R, Iwahana T, Kato H, et al.

International journal of cardiology. Heart & vasculature 2021; (37()):100915 doi:10.1016/j.ijcha.2021.100915.

Clinical analysis of hypereosinophilic syndrome first presenting with asthma-like symptoms.

Wei X, Li X, Wei Z, et al.

Annals of medicine 2022; (54(1)):11-21 doi:10.1080/07853890.2021.2014555.

Loeffler endocarditis with intracardiac thrombus: case report and literature review.

Zhang Q, Si D, Zhang Z, Zhang W

BMC cardiovascular disorders 2021; (21(1)):615 doi:10.1186/s12872-021-02443-2.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.

Grayson PC, Ponte C, Suppiah R, et al.

Annals of the rheumatic diseases 2022; (81(3)):309-314 doi:10.1136/annrheumdis-2021-221794.

The Neutrophil/Lymphocyte Ratio is an Independent Predictor of All-Cause Mortality in Patients with Idiopathic Hypereosinophilic Syndrome.

Xue J, Jiang J, Liu Y

Journal of inflammation research 2022; (15()):1899-1906 doi:10.2147/JIR.S357758.

Hypereosinophilic syndrome presenting as acute ischemic stroke, myocardial infarction, and arterial involvement: A case report.

Sun RR, Chen TZ, Meng M

World journal of clinical cases 2022; (10(11)):3547-3552 doi:10.12998/wjcc.v10.i11.3547.

Updates on eosinophilic disorders.

Tzankov A, Reichard KK, Hasserjian RP, et al.

Virchows Archiv : an international journal of pathology 2023; (482(1)):85-97 doi:10.1007/s00428-022-03402-8.

Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis.

Takeuchi H, Kawamura K, Kawasaki T, Oka N

Frontiers in neurology 2022; (13()):1057767 doi:10.3389/fneur.2022.1057767.

Approach to the patient with suspected hypereosinophilic syndrome.

Klion AD

Hematology. American Society of Hematology. Education Program 2022; (2022(1)):47-54 doi:10.1182/hematology.2022000367.

Idiopathic Hypereosinophilia: A Multicenter Retrospective Analysis.

Rhyou HI, Lee SE, Kim MY, et al.

Journal of asthma and allergy 2022; (15()):1763-1771 doi:10.2147/JAA.S388341.

Clinical and histopathological features of hypereosinophilic syndrome with cutaneous involvement: The Mayo Clinic Experience.

Zayas J, Peters MS, Butterfield JH, et al.

Journal of cutaneous pathology 2023; (50(5)):455-465 doi:10.1111/cup.14410.

[Eosinophilia: hypereosinophilic syndrome vs. eosinophilic granulomatosis with polyangiitis].

Holle JU, Moosig F

Zeitschrift fur Rheumatologie 2023; (82(4)):307-320 doi:10.1007/s00393-023-01345-2.

French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes.

Groh M, Rohmer J, Etienne N, et al.

Orphanet journal of rare diseases 2023; (18(1)):100 doi:10.1186/s13023-023-02696-4.

The international consensus classification of eosinophilic disorders and systemic mastocytosis.

Wang SA, Orazi A, Gotlib J, et al.

American journal of hematology 2023; (98(8)):1286-1306 doi:10.1002/ajh.26966.

Idiopathic Hypereosinophilic Syndrome: A Case Report.

Agudo M, Santos F, Teixeira Reis A, et al.

Cureus 2023; (15(6)):e39964 doi:10.7759/cureus.39964.

Low-dose anti-IL 5 treatment in idiopathic hypereosinophilic syndrome: towards a precision medicine approach for remission maintenance.

Caminati M, Maule M, Benoni R, et al.

Orphanet journal of rare diseases 2023; (18(1)):302 doi:10.1186/s13023-023-02918-9.

Hypereosinophilia causes progressive cardiac pathologies in mice.

Diny NL, Wood MK, Won T, et al.

iScience 2023; (26(10)):107990 doi:10.1016/j.isci.2023.107990.

Biologic therapy in rare eosinophil-associated disorders: remaining questions and translational research opportunities.

Khoury P, Roufosse F, Kuang FL, et al.

Journal of leukocyte biology 2024; (116(2)):307-320 doi:10.1093/jleuko/qiae051.

Co-occurrence of Idiopathic Hypereosinophilic Syndrome in End-Stage Renal Disease Patients Undergoing Maintenance Hemodialysis.

Elbahoty M, Elnaggar S, Soror N, et al.

Cureus 2024; (16(2)):e53758 doi:10.7759/cureus.53758.

Hypereosinophilic syndrome associated with multiple thromboses requiring ICU admission: A case report.

Ouanes I, Toumi S, Ben Cheikh Y, et al.

La Tunisie medicale 2023; (101(10)):783-786.

World Health Organization and International Consensus Classification of eosinophilic disorders: 2024 update on diagnosis, risk stratification, and management.

Shomali W, Gotlib J

American journal of hematology 2024; (99(5)):946-968 doi:10.1002/ajh.27287.

Involvement of the JAK-STAT pathway in the molecular landscape of tyrosine kinase fusion-negative hypereosinophilic syndromes: A nationwide CEREO study.

Groh M, Fenwarth L, Labro M, et al.

American journal of hematology 2024; (99(6)):1108-1118 doi:10.1002/ajh.27306.

Hypereosinophilic syndrome: a rare cause of ST-elevation myocardial infarction and thrombus formation on the aortic valve.

Jørgensen MD, Schneider IR, Thomsen GN, Dahl JS

BMJ case reports 2024; (17(4)) doi:10.1136/bcr-2023-259494.

Prognostic factors of idiopathic hypereosinophilic syndrome: A nationwide survey in Japan.

Honda A, Masuda Y, Oyama Y, et al.

British journal of haematology 2024; (205(3)):967-977 doi:10.1111/bjh.19527.

The three stages of eosinophilic cardiac damage: A series of case reports.

Ferreira J, Gonçalves S, Duarte T, et al.

Journal of cardiology cases 2024; (30(1)):5-8 doi:10.1016/j.jccase.2024.02.013.

A case report of right-sided heart failure secondary to JAK2-mutation Loeffler endocarditis.

Liu X, Liu L, Yan Z, et al.

ESC heart failure 2024; (11(6)):4413-4419 doi:10.1002/ehf2.15015.

Idiopathic hypereosinophilic syndromes and rare dysimmune conditions associated with hyper-eosinophilia in practice: An innovative multidisciplinary approach.

Caminati M, Carpagnano LF, Alberti C, et al.

The World Allergy Organization journal 2024; (17(8)):100928 doi:10.1016/j.waojou.2024.100928.

Imatinib is effective in some PDGFRA/B-negative hypereosinophilic syndromes: A step closer to unveiling underlying mechanisms.

Loscocco GG, Helbig G

British journal of haematology 2024; (205(6)):2136-2138 doi:10.1111/bjh.19853.

Multiple Cerebral Infarcts and Encephalopathy as the First Clinical Manifestations of Hypereosinophilic Syndrome: A Case Report and Narrative Review.

Romano S, Avola G, Angeli MC, et al.

Pulse (Basel, Switzerland) 2024; (12(1)):106-112 doi:10.1159/000539379.

Cardiac Abnormalities in Hypereosinophilic Syndromes.

Hotta VT, Nastari RR, Oishi GDSL, et al.

Arquivos brasileiros de cardiologia 2024; (121(10)):e20240190 doi:10.36660/abc.20240190.

Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort.

Lefèvre G, Bleuse S, Puyade M, et al.

Allergy 2025; (80(4)):1100-1110 doi:10.1111/all.16463.

Single-center off-label benralizumab use for refractory hypereosinophilic syndrome demonstrates satisfactory safety and efficacy.

Veltman Y, Aalbers AM, Hermans MAW, Mutsaers PGNJ

EJHaem 2025; (6(1)):e1014 doi:10.1002/jha2.1014.

Refractory Idiopathic Hypereosinophilic Syndrome Presenting with Myocarditis and Responding to Imatinib: A Case Report.

Albayyat RA, AlQahtani SY, Sharofna KA

Saudi journal of medicine & medical sciences 2025; (13(1)):68-72 doi:10.4103/sjmms.sjmms_503_23.

Evidence of inequities experienced by the rare disease community with respect to receipt of a diagnosis and access to services: a scoping review of UK and international evidence.

Briscoe S, Martin Pintado C, Sutcliffe K, et al.

Orphanet journal of rare diseases 2025; (20(1)):303 doi:10.1186/s13023-025-03818-w.

Case Report: Idiopathic hypereosinophilic syndrome presenting with gastrointestinal involvement mimicking IBD: a diagnostic challenge.

Amer J, Noori F, Hamdan D, et al.

Frontiers in medicine 2025; (12()):1595193 doi:10.3389/fmed.2025.1595193.

Organ-Restricted Hypereosinophilic Syndrome: A Case of Recurrent Hypereosinophilic Myopericarditis.

Alhajri N, Fadlelseed S, Abokhater N, et al.

JACC. Case reports 2025; (30(35)):105535 doi:10.1016/j.jaccas.2025.105535.

Idiopathic hypereosinophilic syndrome complicated by computed tomography-negative cerebral infarction, strangulated intestinal obstruction, and hepatic portal venous gas: a case report of multi-system involvement.

Wei H, Ji C, Dong F, et al.

Quantitative imaging in medicine and surgery 2025; (15(11)):11630-11641 doi:10.21037/qims-2025-1070.

Loffler endocarditis as an initial manifestation of lung adenocarcinoma: A case report.

Yao H, Chen Y, Wu C, et al.

Medicine 2025; (104(50)):e46394 doi:10.1097/MD.0000000000046394.

Fulminant Idiopathic Hypereosinophilic Syndrome Presenting with Cardiac, Neurologic and Haematologic Involvement.

Ibrahim N, Batri A, Rivera RR, Zwein A

European journal of case reports in internal medicine 2025; (12(12)):006010 doi:10.12890/2025_006010.

From hypereosinophilia to hypereosinophilic syndrome: real-world application of a two-tailed approach for HES diagnosis.

Nicola S, Borrelli R, Ridolfi I, et al.

Frontiers in immunology 2025; (16()):1735131 doi:10.3389/fimmu.2025.1735131.

Exploring the Intersection of Rare Diseases and Mental Health Within the Diagnostic Odyssey: A Narrative Review and Thematic Synthesis.

Wu E, Isobel S, Beckett P

Nursing open 2026; (13(4)):e70488 doi:10.1002/nop2.70488.

Coronary vasospasm in a patient with RUNX1 mutation in eosinophilic leukemia and hypereosinophilic syndrome: A case report.

AlShamrani AA, Alqaraishi AM, Almukhlifi AS, et al.

Journal of cardiology cases 2026; (33(4)):135-138 doi:10.1016/j.jccase.2026.01.001.

Pro-inflammatory and counter-regulatory modulators of interleukin-5-driven eosinophil programs: a framework for precision medicine in eosinophilic diseases.

Sasaki H, Miyata J, Fukunaga K

Frontiers in immunology 2026; (17()):1836661 doi:10.3389/fimmu.2026.1836661.

Hypereosinophilia of persistent/unknown cause: a bone marrow-based study integrating WHO-HAEM5 criteria and myeloid next-generation sequencing.

Eletrebi MM, Sanchez-Ramirez J, Abdalla A, Holman CJ

American journal of clinical pathology 2026; (165(5)) doi:10.1093/ajcp/aqag031.

Severe Persistent Hypereosinophilia of Undetermined Significance: Diagnostic Challenges in Clinical Practice and Two-Year Follow-Up.

Leru PM, Anton VF, Georgescu DG

Romanian journal of internal medicine = Revue roumaine de medecine interne 2026; doi:10.2478/rjim-2026-0014.

Organ Involvement and Constitutional Symptoms in Idiopathic Hypereosinophilic Syndrome: A 72-Patient Single-Centre Cohort.

Łacwik P, Kupryś-Lipińska I, Kucharczyk A, et al.

Journal of clinical medicine 2026; (15(14)) doi:10.3390/jcm15145569.

Paraneoplastic Hypereosinophilia With Multisystem Involvement as an Atypical Presentation of Lung Adenocarcinoma.

Bravo S, Machado A, Medeiros P, et al.

Cureus 2026; (18(8)):e114369 doi:10.7759/cureus.114369.

Hypereosinophilic syndrome with Löffler endocarditis and multiple cerebral infarctions in a patient with a CLL-phenotype clonal B-cell population: a case report.

Wang H, Zhu F, Hu H

Frontiers in cardiovascular medicine 2026; (13()):1924869 doi:10.3389/fcvm.2026.1924869.

Beyond eosinophil counts: organ involvement patterns across hypereosinophilic syndrome subtypes in a single-center cohort.

Nicola S, Negrini S, Lo Sardo L, et al.

Frontiers in immunology 2026; (17()):1933052 doi:10.3389/fimmu.2026.1933052.