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[Hypereosinophilic syndromes].

Matthieu Groh1, Guillaume Lefèvre2, Félix Ackermann1

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Hypereosinophilic syndromes (HES) involve chronic eosinophilia causing organ damage. Treatment varies by subtype, with targeted therapies showing promise for some HES forms.

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Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Hypereosinophilic syndromes (HES) are characterized by persistent high eosinophil counts, leading to diverse organ damage.
  • HES encompasses neoplastic (HESN) and reactive (HESR) subtypes, with a significant portion remaining of undetermined cause.
  • Clinical manifestations are varied, with cardiac, dermatological, respiratory, and gastrointestinal systems frequently affected.

Purpose of the Study:

  • To define Hypereosinophilic Syndromes (HES) and their classification.
  • To outline the clinical manifestations and prognostic factors in HES.
  • To review current and emerging therapeutic strategies for HES subtypes.

Main Methods:

  • Literature review of HES classification, clinical features, and treatment options.
  • Analysis of diagnostic criteria including blood eosinophilia thresholds and organ damage.
  • Summarization of treatment modalities, including targeted therapies and clinical trial outcomes.

Main Results:

  • HES is defined by eosinophilia ≥ 1.5 G/L for over a month, causing organ damage.
  • Subtypes include neoplastic (HESN) and reactive (HESR), with HESL as a lymphoid variant; ~50% of cases are of unknown etiology.
  • Cardiac involvement is a key prognostic factor, and HESN/HESL carry a risk of transformation into hematological malignancies.

Conclusions:

  • HES requires careful etiological workup and classification into subtypes for appropriate management.
  • Prognosis is significantly influenced by cardiac involvement and malignant transformation risk.
  • Targeted therapies, particularly IL-5 blockade, offer promising avenues for refractory HES, though access is currently limited.