Orphanet Journal of Rare Diseases (Apr 2023)

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

  • Matthieu Groh,
  • Julien Rohmer,
  • Nicolas Etienne,
  • Wadih Abou Chahla,
  • Antoine Baudet,
  • Aurélie Chan Hew Wai,
  • Cécile Chenivesse,
  • Irena Clisson Rusek,
  • Vincent Cottin,
  • Matthieu Decamp,
  • Pascal De Groote,
  • Fanny Delahousse,
  • Nicolas Duployez,
  • Stanislas Faguer,
  • Frédéric Gottrand,
  • Florent Huang,
  • Thierry Leblanc,
  • Antoine Magnan,
  • Thierry Martin,
  • Geoffrey Mortuaire,
  • Antoine Néel,
  • Luc Paris,
  • Arnaud Petit,
  • Julien Rossignol,
  • Nicolas Schleinitz,
  • Juliette Soret-Dulphy,
  • Delphine Staumont-Salle,
  • Benjamin Terrier,
  • Louis Terriou,
  • Jean-François Viallard,
  • Guillaume Lefèvre,
  • Jean-Emmanuel Kahn

DOI
https://doi.org/10.1186/s13023-023-02696-4
Journal volume & issue
Vol. 18, no. 1
pp. 1 – 35

Abstract

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Abstract 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). Here, thanks to an extensive literature review, and thanks to the joint work of a large panel of experts involving physicians from both adult and pediatric medicine and from various subspecialties (as well as a representative of a patients’ association representative), we provide recommendations for both the step-by step diagnostic workup of eosinophilia (whether unexplained or within specific contexts) as well as the management and follow-up of the full spectrum of eosinophilic disorders (including clonal, reactive, lymphocytic and idiopathic HES, as well as single-organ diseases). Didactic prescription summaries intended to facilitate the prescription of eosinophil-targeted drugs are also provided, as are practical diagnostic and therapeutic algorithms. Lastly, this set of recommendations also includes a summary intended for general practitioners, as well as an overview of the therapeutic patient education program set up by the French reference center for HES. Further updates will be mandatory as new validated information emerges.

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