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. 2025 Sep 22:S2213-2198(25)00903-1.
doi: 10.1016/j.jaip.2025.09.009. Online ahead of print.

Development and validation of the FIP-Score for the screening of FIP1L1::PDGFRA-associated hypereosinophilic syndrome

Romain Stammler  1 , Alexandre Vallée  2 , Julien Rohmer  3 , Nathalie Grardel  4 , Noémie Abisror  5 , Wadih Abou Chahla  6 , Félix Ackermann  1 , Antoine Baudet  7 , Nabil Belfeki  8 , Raouf Ben Abdelali  9 , Audrey Bidet  10 , Jean-Sébastien Bladé  11 , Séverine Bleuse  12 , Bernard Bonnotte  13 , Mohammed Azzedine Bouderbala  14 , Jean-Michel Cayuela  15 , Safia Chebrek  16 , Jérémie Dion  17 , Antoine Dossier  18 , Nicolas Duployez  19 , Stanislas Faguer  20 , Pascale Flandrin-Gresta  21 , Lionel Galicier  22 , Catherine Godon  23 , Maximilien Grall  24 , Vincent Jachiet  5 , Mathieu Jouvray  25 , Irina Latu  26 , Emmanuel Ledoult  12 , Etienne Lengline  27 , Amélie Leurs  28 , Ludovic Lhermitte  29 , François Lifermann  30 , Nicolas Limal  31 , Bertrand Lioger  32 , Maxime Lugosi  33 , Irène Machelart  34 , Mickaël Martin  35 , Nihal Martis  36 , Laurent Mauvieux  37 , Sara Melboucy-Belkhir  38 , Catherine Mohr  39 , Guillaume Moulis  40 , Marie Joelle Mozicconacci  41 , Dina Naguib  42 , Antoine Néel  43 , Franck E Nicolini  44 , Roderau Outh  45 , Kewin Panel  46 , Claude Preudhomme  47 , Mathieu Puyade  48 , Thomas Quemeneur  49 , Viviane Queyrel Moranne  50 , Jérôme Rey  51 , Philippe Rousselot  52 , Nicolas Schleinitz  22 , Borhane Slama  16 , Delphine Staumont-Salle  53 , Camille Taillé  54 , Louis Terriou  12 , Ludovic Tréfond  55 , Jean François Viallard  56 , Jean-Emmanuel Kahn  57 , Guillaume Lefèvre  58 , Matthieu Groh  59 ; COHESion, CEREO and GBMHM groups
Collaborators, Affiliations

Development and validation of the FIP-Score for the screening of FIP1L1::PDGFRA-associated hypereosinophilic syndrome

Romain Stammler et al. J Allergy Clin Immunol Pract. .

Abstract

Background: FIP1L1-PDFGRA (F/P)-associated Hypereosinophilic Syndrome (HES) is a rare condition. F/P fusion gene testing is one of the first-line investigations in patients with unexplained eosinophilia and yields poor diagnostic performance.

Objective: To build and validate the FIP-Score: a set of weighted criteria warranting testing for the F/P fusion gene.

Methods: We merged data from 151 patients with F/P-associated HES and 320 patients with either F/P-negative HES (n=279) or hypereosinophilia of undetermined significance (n=41). Training and validation cohorts (comprising respectively 90% and 10% of all patients) were randomly dichotomized. Variables with a p-value <0.20 in univariate analysis were included in the multivariable forward-backward logistic regression model to assess their independent contribution to testing positive for the F/P fusion gene. Beta coefficients from multivariable logistic regression were used to assign points for the construction of the score.

Results: Age under 66 years, male sex, splenomegaly, lymphomatoid papulosis, absence of gastrointestinal involvement, high serum vitamin B12, high serum tryptase and normal serum immunoglobulin E levels were the eight variables retained in the model. The best cutoff value was greater than 48. The model yielded a sensitivity, specificity, positive predictive value, negative predictive value and area under the curve respectively of 88.3%, 93.7%, 87.1%, 94.4%, and 0.962 in the training dataset and of 85.7%, 97.0%, 85.7% and 97.0%, 0.986 in the validation dataset.

Conclusion: The FIP-score highlights the need for closely selecting patients with hypereosinophilia for whom F/P fusion gene testing should be performed, resulting in medical time reduction and substantial cost-savings.

Keywords: Diagnostic test; Eosinophilia; FIP1L1-PDGFRA fusion protein; Hypereosinophilic Syndrome; Myeloid/Lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions.

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