DOI: 10.12890/2026_007269

Lymphocytic variant hypereosinophilic syndrome overlaps with angioimmunoblastic T-cell lymphoma: a case report and literature review

Authors

  • Nabil Belfeki Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud Ile de France, Melun, France
  • Omaima El Koudssi Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud Ile de France, Melun, France
  • Pierre Isnard Department of Pathology and Cytology, Assistance Publique-Hôpitaux de Paris, Necker Hospital, Paris, France
  • Guillaume Lefevre Institute of Immunology, Department of Internal Medicine and Clinical Immunology, Lille University Hospital; Centre de Référence des Syndromes Hyperéosinophiliques (CEREO), Suresnes, France

omaima.elkoudssi@gmail.com

Keywords

Eosinophilia, adenomegaly, lymphoproliferation, flow cytometry

Abstract

Introduction: Lymphocytic-variant of hypereosinophilic syndrome (L-HES) is a clonal T-cell disorder driven by an aberrant Th2 response, typically featuring CD3- CD4+ T-cell expansion, eosinophil-mediated tissue injury, most commonly skin lesions, and lymphadenopathy. Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma of follicular helper T-cell origin; it can present with similar clinical features such as lymphadenopathy, rash and eosinophilia. Histological and immunophenotypic features may also be common in both diseases, thus creating a diagnostic overlap.
Case description: We report the case of a 53-year-old man with a medical history of allergic rhinitis who developed a diffuse, persistent maculopapular rash with pruritis. Physical examination revealed inguinal lymphadenopathy without organomegaly, laboratory investigations demonstrated marked eosinophilia, and hypergammaglobulinemia, while additional testing excluded any infectious or autoimmune aetiologies. Lymphocytic immunophenotyping on peripheral blood and skin biopsy revealed a high proportion of CD3-CD4+ cells exhibiting a classic TH2 phenotype, consistent with L-HES. However, lymph node histology demonstrated architectural remodelling with intrafollicular infiltration by atypical T lymphocytes showing a T-cell follicular helper phenotype, findings indicative of AITL. The coexistence of L-HES and AITL was established. The patient underwent polychemotherapy, resulting in rapid clinical improvement of skin lesions with sustained remission at 6 months of follow up.
Conclusion: L-HES and AITL may coexist, or L-HES may precede overt lymphoma, suggesting clonal evolution within a shared biological spectrum. This case highlights diagnostic pitfalls and emphasizes the need for integrated clinical, pathological, flow cytometric and molecular evaluation.

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References

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Published

2026-08-12

Issue

Vol. 13 No. 9 (2026)

Categories

How to Cite

1.
Belfeki N, El Koudssi O, Isnard P, Lefevre G. Lymphocytic variant hypereosinophilic syndrome overlaps with angioimmunoblastic T-cell lymphoma: a case report and literature review. EJCRIM [Internet]. 2026 Aug. 12 [cited 2026 Nov. 3];13(9). Available from: https://ejcrim.com/index.php/ejcrim/article/view/7269

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