Development and Validation of the FIP Score for the Screening of FIP1L1::PDGFRA-Associated Hypereosinophilic Syndrome

Romain Stammler¹, Alexandre Vallée², Julien Rohmer³, Nathalie Grardel⁴, Noémie Abisror⁵, Wadih Abou Chahla⁶, Félix Ackermann¹, Antoine Baudet⁷, Nabil Belfeki⁸, Raouf Ben Abdelali⁹, Audrey Bidet¹⁰, Jean-Sébastien Bladé¹¹, Séverine Bleuse¹², Bernard Bonnotte¹³, Mohammed Azzedine Bouderbala¹⁴, Jean-Michel Cayuela¹⁵, Safia Chebrek¹⁶, Jérémie Dion¹⁷, Antoine Dossier¹⁸, Nicolas Duployez¹⁹, Stanislas Faguer²⁰, Pascale Flandrin-Gresta²¹, Lionel Galicier²², Catherine Godon²³, Maximilien Grall²⁴, Vincent Jachiet⁵, Mathieu Jouvray²⁵, Irina Latu²⁶, Emmanuel Ledoult¹², Etienne Lengline²⁷, Amélie Leurs²⁸, Ludovic Lhermitte²⁹, François Lifermann³⁰, Nicolas Limal³¹, Bertrand Lioger³², Maxime Lugosi³³, Irène Machelart³⁴, Mickaël Martin³⁵, Nihal Martis³⁶, Laurent Mauvieux³⁷, Sara Melboucy-Belkhir³⁸, Catherine Mohr³⁹, Guillaume Moulis⁴⁰, Marie Joelle Mozicconacci⁴¹, Dina Naguib⁴², Antoine Néel⁴³, Franck E Nicolini⁴⁴, Roderau Outh⁴⁵, Kewin Panel⁴⁶, Claude Preudhomme⁴⁷, Mathieu Puyade⁴⁸, Thomas Quemeneur⁴⁹, Viviane Queyrel Moranne⁵⁰, Jérôme Rey⁵¹, Philippe Rousselot⁵², Nicolas Schleinitz²², Borhane Slama¹⁶, Delphine Staumont-Salle⁵³, Camille Taillé⁵⁴, Louis Terriou¹², Ludovic Tréfond⁵⁵, Jean François Viallard⁵⁶, Jean-Emmanuel Kahn⁵⁷, Guillaume Lefèvre⁵⁸, Matthieu Groh⁵⁹; COHESion, CEREO and GBMHM groups

Collaborators, Affiliations

Collaborators

COHESion, CEREO and GBMHM groups:
Sébastien Abad, Henri Abi Rached, Amir Adedjouma, Didier Adiko, Christian Agard, Kais Ahmad, Nuri Ahwij, Jean-Christophe Allo, Zineb Amrani, Achille Aouba, Hélène Aubert, Nathalie Baize, André Baruchel, Caroline Beal, Issam Belhaj, Maxime Bemba, Alice Berezne, Clément Beuvon, Marie Beylot Barry, Odile Beyne Raury, Philip Bielefeld, Constance Blandin, Mario Boisseau, Caroline Bonmati, Cécile Borel, Daoud Bouredji, Hélène Boutroux, Anne Brignier, Benoit Brihaye, Marc Brouillard, Thomas Brunet, Claude-Eric Bulabois, France Campos, Martin Carre, Pascal Cathébras, Emilie Cayssials, Emilie Chalayer, Cécile Chenivesse, Jean Claude Chomel, Valérie Coiteux, Thibaut Comont, Adrien Contejean, Pascale Cony-Makhoul Anne Corby, Vincent Cottin, Amélie Couteau Chardon, Priscille Couture, Yoann Crabol, Gwenvael Danic, Adrien Daniel, Stéphane Darre, Sebastien De Almeida, Adèle De Masson, Eric Delabesse, Thomas Delaye, Fréderic Dezoteux, Viviane Dubruille, Clairelyne Dupin, Mylène Duplan, Cécile Audrey Durel, Mikaël Ebbo, Japhete Darline Elenga Koenga, Aberrazak El Yamani, Alexandra Espitia, Pascaline Etancelin, Gabriel Etienne, Nicolas Etienne, Marine Eustache, Philippe Evon, Olivier Fain, Sophie Georgin Lavialle, Bertrand Godeau, Cécile Golden, Tiphaine Goulenok, Clément Gourguechon, Carine Greib, Aurélien Guffroy, Philippe Guilpain, Emmanuel Gyan, Eric Hachulla, Marion Hermet, Françoise Huguet, Mathilde Hunault-Berger Jean Christophe Ianotto, Lucas Inchiappa, Ibrahim Jdid, Elisa Joly, Katayoun Jondeau, Morgane Joubert, Anne Jourdain, Mathieu Jouvray, Galith Kalmi, Brigitte Lagrange, Judith Landman-Parker, Mathilde Le Brun, Noémie Le Gouellec, Caroline Lejeune, Sarah Le Naour, Mathilde Leclercq, Paul Legendre, Claire Le Pendu, Faustine Lhomme, Olivier Lidove, Benoit Lohberger-Timsit Naelle Lombion, Elena Lopinet, Pierre Lozach, Aurélie Madelon, Léa Mairot, Fréderic Maloisel, Anne Marfaing, Victor Margelidon, Ibrahim Marroun, Agathe Masseau, François Maurier, Arsène Mekinian, Mathieu Meunier, Marc Michel, Chafika Morati-Hafsaoui, Marjolaine Morgand, Emilie Muller, Thierry Muron, Alexandre Nguyen, Hubert Nielly, Mario Ojeda, Thomas Papo, Romain Paule, Arnaud Petit, Jean Michel Pignon, Camille Prot-Leurent, Jérome Razanamahery, Sabine Revuz, Sophie Rigaudeau, Etienne Riviere, Cédric Rossi, Manon Roucoules, Jean-Philippe Rougier, Mathilde Roumier, Benedicte Rouvière, Frédéric Roy Peaud, Arnaud Saint Lezer, Laurent Sailler, Cécile Saint-Pastou Sébastien Sanges, Gaetan Sauvetre, Léa Savey, Quentin Scanvion, Laurent Schwarb, Damien Sene, Laurence Simon, Bohrane Slama, Micha Srour, Felipe Suarez, Benjamin Subran, Suzanne Tavitian, Colas Tcherakian, Nathalie Tieulie, Jose Miguel Torregrosa, Elise Toussaint, Julie Trichereau, Rémi Vatan, Gael Vaudour, Alexandre Vlakos, Sorin Visanica, Laurent Voilat, Fanny Vuotto, Stéphanie Wanin, Lidwine Stervinou-Wemeau Juliette Woessner, Hacène Zerazhi, Jean Marc Zini, Stéphane Zographos

Affiliations

¹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Department of Internal Medicine, Foch Hospital, Suresnes, France.
² Department of Epidemiology-Data-Biostatistics, Delegation of Clinical Research and Innovation (DRCI), Foch Hospital, Suresnes, France.
³ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Sorbonne Paris Cité University, Assistance Publique Hôpitaux de Paris, Internal Medicine Department, Bichat hospital, Paris, France.
⁴ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Hematology Institute, Lille, France.
⁵ Internal Medicine Department, Sorbonne University, Saint Antoine Hospital, Assistance Publique Hôpitaux de Paris, Paris, France.
⁶ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Pediatric Hematology Department, University Hospital Center of Lille, Lille, France.
⁷ Internal Medicine Department, Hospital Center of Annecy, Annecy, France.
⁸ Internal Medicine and Clinical Immunology Department, Sud Ile de France Group Hospital, Melun, France.
⁹ Pole of Hematology and Oncology, CERBA Laboratory, Saint Ouen l'Aumône, France.
¹⁰ Laboratory of Hematology, CHU de Bordeaux, Pessac, France.
¹¹ Hôpital d'Instruction des Armées Saint Anne, Internal Medicine and Oncology Department, Boulevard Saint Anne, Toulon, France.
¹² National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Immunology Institute, Internal Medicine and Clinical Immunology Department, University Hospital Center of Lille, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France.
¹³ Service de Médecine Interne et Immunologie Clinique, Centre Hospitalo-Universitaire Dijon Bourgogne, Université de Bourgogne, Institut national de la santé et de la recherche médicale unité mixte de recherche 1098, Dijon, France.
¹⁴ Service d'Hématologie et Oncologie Médicale, Centre Hospitalier de Poissy Saint-Germain, Poissy, France.
¹⁵ Laboratory of Hematology, Saint Louis Hospital, University Paris Cité, Paris, France.
¹⁶ Hematology Department, H. Duffaut Hospital Center, Avignon, France.
¹⁷ Internal Medicine and clinical Immunopathology Department, University Hospital Center of Toulouse, Pole Institut Universitaire du Cancer Oncopole University Hospital Center, Toulouse, France.
¹⁸ Sorbonne Paris Cité University, Assistance Publique Hôpitaux de Paris, Internal Medicine Department, Bichat hospital, Paris, France.
¹⁹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Hematology Institute, Lille, France; University of Lille, Centre National de la Recherche Scientifique, INSERM U1277, Cancer Heterogeneity, Plasticity, and Resistance to Therapies (CANTHER), Lille, France.
²⁰ Department of Nephrology and Organ Transplantation, National Reference Center for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, France; INSERM U1297 (I2MC), F-31000 Toulouse, France.
²¹ Hematology Laboratory, Nord Hospital, University Hospital Center of Saint Etienne, Saint Etienne, France.
²² Internal Medicine Department, CHU La Timone, AP-HM, Marseille, France.
²³ Hematological Cytogenetic Laboratory, University Hospital Center of Nantes, Nantes, France.
²⁴ Internal Medicine Department, University Hospital Center of Rouen, Rouen, France.
²⁵ Internal Medicine Department, Hospital Center of Arras, Arras, France.
²⁶ Pneumology Department, H. Duffaut Hospital Center, Avignon, France.
²⁷ Department of Hematology, Saint Louis Hospital, Paris, France.
²⁸ Internal Medicine Department, Alexandra Lepève Hospital Center, Dunkerque, France.
²⁹ University of Paris, Institut National de Recherche Médicale U1151, Laboratory of Onco-Hematology, Hôpital Necker Enfants-Malades, Paris, France.
³⁰ Internal Medicine Department, Hospital Center of Dax, Dax, France.
³¹ Internal Medicine Department, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris-Est Créteil University, Créteil, France.
³² Internal and Polyvalent Medicine Department, Hospital Center of Blois, Blois, France.
³³ Internal Medicine Department, University Grenoble Alpes, University Hospital Center Grenoble Alpes, Grenoble, France.
³⁴ Internal Medicine Department, Hospital Center of Côte Basque, Bayonne, France.
³⁵ Internal Medicine Department, University Hospital Center La Milétrie, INSERM U1313, University of Poitiers, Poitiers, France.
³⁶ Internal Medicine Department, Nice University Hospital Center, Nice, France.
³⁷ Hematology Department, University Hospital Center of Strasbourg, University of Strasbourg, CNRS UPR3572, Strasbourg, France.
³⁸ Internal and Polyvalent Medicine Department, Saint Quentin Hospital Center, Saint Quentin, France.
³⁹ Hematology and Oncology Department, Hospital Group Sud Réunion, Saint Pierre, Réunion, France.
⁴⁰ Internal Medicine Department, University Hospital Center of Toulouse, Clinical Investigation Center 1436, PEPSS Team, Toulouse, France.
⁴¹ Paoli-Calmettes Institute, Cancerology Research Center of Marseille, Biopathology, Marseille, France.
⁴² Laboratory of Hematology, University Hospital Center of Caen, Caen, France.
⁴³ Internal Medicine Department, University Hospital Center of Nantes, Nantes, France.
⁴⁴ Cancerology and Hematology Department, Léon Bérard Center, Lyon, France.
⁴⁵ Internal Medicine Department, Hospital Center of Perpignan, Perpignan, France.
⁴⁶ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France.
⁴⁷ University Hospital Center of Lille, Hematology Institute, Lille, France.
⁴⁸ Internal Medicine Department, University Hospital Center la Milétrie, CICP-1042, Poitiers, France.
⁴⁹ Nephrology and Internal Medicine Department, Hospital Center of Valenciennes, Valenciennes, France.
⁵⁰ Rheumatology Department, University Hospital Center L'Archet, Nice, France.
⁵¹ Department of Hematology, Institut Paoli-Calmettes, Marseille, France.
⁵² Hematology Department, Versailles André Mignot Hospital, University Paris-Saclay, Le Chesnay, France.
⁵³ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Dermatology Department, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France.
⁵⁴ Pneumology Department and Referal Center for Rare Pulmonary Diseases, Bichat Hospital, Assistance Publique Hôpitaux de Paris, UMR 1152, Paris Cité University, Paris, France.
⁵⁵ Internal Medicine Department, University Hospital Center of Clermont Ferrand, Gabriel Montpied Hospital, INSERM U1071, INRA USC2018, M2iSH, Clermont Auvergne University, Clermont Ferrand, France.
⁵⁶ Internal Medicine Department, University Hospital Center of Bordeaux, Bordeaux, France.
⁵⁷ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Internal Medicine Department, Ambroise Paré Hospital, AP-HP. 9, Boulogne, France; Infection and Inflammation, UMR 1173, INSERM, UVSQ/Paris Saclay University, Montigny-le-Bretonneux, France.
⁵⁸ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Immunology Institute, Internal Medicine and Clinical Immunology Department, University Hospital Center of Lille, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France; University Lille, CHU Lille, INSERM, U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France.
⁵⁹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Department of Internal Medicine, Foch Hospital, Suresnes, France; University Lille, CHU Lille, INSERM, U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France. Electronic address: m.groh@hopital-foch.com.

PMID: 40992688
DOI: 10.1016/j.jaip.2025.09.009

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. 2025.

. 2025 Sep 22:S2213-2198(25)00903-1.

doi: 10.1016/j.jaip.2025.09.009. Online ahead of print.

Authors

Collaborators

COHESion, CEREO and GBMHM groups:
Sébastien Abad, Henri Abi Rached, Amir Adedjouma, Didier Adiko, Christian Agard, Kais Ahmad, Nuri Ahwij, Jean-Christophe Allo, Zineb Amrani, Achille Aouba, Hélène Aubert, Nathalie Baize, André Baruchel, Caroline Beal, Issam Belhaj, Maxime Bemba, Alice Berezne, Clément Beuvon, Marie Beylot Barry, Odile Beyne Raury, Philip Bielefeld, Constance Blandin, Mario Boisseau, Caroline Bonmati, Cécile Borel, Daoud Bouredji, Hélène Boutroux, Anne Brignier, Benoit Brihaye, Marc Brouillard, Thomas Brunet, Claude-Eric Bulabois, France Campos, Martin Carre, Pascal Cathébras, Emilie Cayssials, Emilie Chalayer, Cécile Chenivesse, Jean Claude Chomel, Valérie Coiteux, Thibaut Comont, Adrien Contejean, Pascale Cony-Makhoul Anne Corby, Vincent Cottin, Amélie Couteau Chardon, Priscille Couture, Yoann Crabol, Gwenvael Danic, Adrien Daniel, Stéphane Darre, Sebastien De Almeida, Adèle De Masson, Eric Delabesse, Thomas Delaye, Fréderic Dezoteux, Viviane Dubruille, Clairelyne Dupin, Mylène Duplan, Cécile Audrey Durel, Mikaël Ebbo, Japhete Darline Elenga Koenga, Aberrazak El Yamani, Alexandra Espitia, Pascaline Etancelin, Gabriel Etienne, Nicolas Etienne, Marine Eustache, Philippe Evon, Olivier Fain, Sophie Georgin Lavialle, Bertrand Godeau, Cécile Golden, Tiphaine Goulenok, Clément Gourguechon, Carine Greib, Aurélien Guffroy, Philippe Guilpain, Emmanuel Gyan, Eric Hachulla, Marion Hermet, Françoise Huguet, Mathilde Hunault-Berger Jean Christophe Ianotto, Lucas Inchiappa, Ibrahim Jdid, Elisa Joly, Katayoun Jondeau, Morgane Joubert, Anne Jourdain, Mathieu Jouvray, Galith Kalmi, Brigitte Lagrange, Judith Landman-Parker, Mathilde Le Brun, Noémie Le Gouellec, Caroline Lejeune, Sarah Le Naour, Mathilde Leclercq, Paul Legendre, Claire Le Pendu, Faustine Lhomme, Olivier Lidove, Benoit Lohberger-Timsit Naelle Lombion, Elena Lopinet, Pierre Lozach, Aurélie Madelon, Léa Mairot, Fréderic Maloisel, Anne Marfaing, Victor Margelidon, Ibrahim Marroun, Agathe Masseau, François Maurier, Arsène Mekinian, Mathieu Meunier, Marc Michel, Chafika Morati-Hafsaoui, Marjolaine Morgand, Emilie Muller, Thierry Muron, Alexandre Nguyen, Hubert Nielly, Mario Ojeda, Thomas Papo, Romain Paule, Arnaud Petit, Jean Michel Pignon, Camille Prot-Leurent, Jérome Razanamahery, Sabine Revuz, Sophie Rigaudeau, Etienne Riviere, Cédric Rossi, Manon Roucoules, Jean-Philippe Rougier, Mathilde Roumier, Benedicte Rouvière, Frédéric Roy Peaud, Arnaud Saint Lezer, Laurent Sailler, Cécile Saint-Pastou Sébastien Sanges, Gaetan Sauvetre, Léa Savey, Quentin Scanvion, Laurent Schwarb, Damien Sene, Laurence Simon, Bohrane Slama, Micha Srour, Felipe Suarez, Benjamin Subran, Suzanne Tavitian, Colas Tcherakian, Nathalie Tieulie, Jose Miguel Torregrosa, Elise Toussaint, Julie Trichereau, Rémi Vatan, Gael Vaudour, Alexandre Vlakos, Sorin Visanica, Laurent Voilat, Fanny Vuotto, Stéphanie Wanin, Lidwine Stervinou-Wemeau Juliette Woessner, Hacène Zerazhi, Jean Marc Zini, Stéphane Zographos

Affiliations

¹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Department of Internal Medicine, Foch Hospital, Suresnes, France.
² Department of Epidemiology-Data-Biostatistics, Delegation of Clinical Research and Innovation (DRCI), Foch Hospital, Suresnes, France.
³ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Sorbonne Paris Cité University, Assistance Publique Hôpitaux de Paris, Internal Medicine Department, Bichat hospital, Paris, France.
⁴ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Hematology Institute, Lille, France.
⁵ Internal Medicine Department, Sorbonne University, Saint Antoine Hospital, Assistance Publique Hôpitaux de Paris, Paris, France.
⁶ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Pediatric Hematology Department, University Hospital Center of Lille, Lille, France.
⁷ Internal Medicine Department, Hospital Center of Annecy, Annecy, France.
⁸ Internal Medicine and Clinical Immunology Department, Sud Ile de France Group Hospital, Melun, France.
⁹ Pole of Hematology and Oncology, CERBA Laboratory, Saint Ouen l'Aumône, France.
¹⁰ Laboratory of Hematology, CHU de Bordeaux, Pessac, France.
¹¹ Hôpital d'Instruction des Armées Saint Anne, Internal Medicine and Oncology Department, Boulevard Saint Anne, Toulon, France.
¹² National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Immunology Institute, Internal Medicine and Clinical Immunology Department, University Hospital Center of Lille, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France.
¹³ Service de Médecine Interne et Immunologie Clinique, Centre Hospitalo-Universitaire Dijon Bourgogne, Université de Bourgogne, Institut national de la santé et de la recherche médicale unité mixte de recherche 1098, Dijon, France.
¹⁴ Service d'Hématologie et Oncologie Médicale, Centre Hospitalier de Poissy Saint-Germain, Poissy, France.
¹⁵ Laboratory of Hematology, Saint Louis Hospital, University Paris Cité, Paris, France.
¹⁶ Hematology Department, H. Duffaut Hospital Center, Avignon, France.
¹⁷ Internal Medicine and clinical Immunopathology Department, University Hospital Center of Toulouse, Pole Institut Universitaire du Cancer Oncopole University Hospital Center, Toulouse, France.
¹⁸ Sorbonne Paris Cité University, Assistance Publique Hôpitaux de Paris, Internal Medicine Department, Bichat hospital, Paris, France.
¹⁹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Hematology Institute, Lille, France; University of Lille, Centre National de la Recherche Scientifique, INSERM U1277, Cancer Heterogeneity, Plasticity, and Resistance to Therapies (CANTHER), Lille, France.
²⁰ Department of Nephrology and Organ Transplantation, National Reference Center for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, France; INSERM U1297 (I2MC), F-31000 Toulouse, France.
²¹ Hematology Laboratory, Nord Hospital, University Hospital Center of Saint Etienne, Saint Etienne, France.
²² Internal Medicine Department, CHU La Timone, AP-HM, Marseille, France.
²³ Hematological Cytogenetic Laboratory, University Hospital Center of Nantes, Nantes, France.
²⁴ Internal Medicine Department, University Hospital Center of Rouen, Rouen, France.
²⁵ Internal Medicine Department, Hospital Center of Arras, Arras, France.
²⁶ Pneumology Department, H. Duffaut Hospital Center, Avignon, France.
²⁷ Department of Hematology, Saint Louis Hospital, Paris, France.
²⁸ Internal Medicine Department, Alexandra Lepève Hospital Center, Dunkerque, France.
²⁹ University of Paris, Institut National de Recherche Médicale U1151, Laboratory of Onco-Hematology, Hôpital Necker Enfants-Malades, Paris, France.
³⁰ Internal Medicine Department, Hospital Center of Dax, Dax, France.
³¹ Internal Medicine Department, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris-Est Créteil University, Créteil, France.
³² Internal and Polyvalent Medicine Department, Hospital Center of Blois, Blois, France.
³³ Internal Medicine Department, University Grenoble Alpes, University Hospital Center Grenoble Alpes, Grenoble, France.
³⁴ Internal Medicine Department, Hospital Center of Côte Basque, Bayonne, France.
³⁵ Internal Medicine Department, University Hospital Center La Milétrie, INSERM U1313, University of Poitiers, Poitiers, France.
³⁶ Internal Medicine Department, Nice University Hospital Center, Nice, France.
³⁷ Hematology Department, University Hospital Center of Strasbourg, University of Strasbourg, CNRS UPR3572, Strasbourg, France.
³⁸ Internal and Polyvalent Medicine Department, Saint Quentin Hospital Center, Saint Quentin, France.
³⁹ Hematology and Oncology Department, Hospital Group Sud Réunion, Saint Pierre, Réunion, France.
⁴⁰ Internal Medicine Department, University Hospital Center of Toulouse, Clinical Investigation Center 1436, PEPSS Team, Toulouse, France.
⁴¹ Paoli-Calmettes Institute, Cancerology Research Center of Marseille, Biopathology, Marseille, France.
⁴² Laboratory of Hematology, University Hospital Center of Caen, Caen, France.
⁴³ Internal Medicine Department, University Hospital Center of Nantes, Nantes, France.
⁴⁴ Cancerology and Hematology Department, Léon Bérard Center, Lyon, France.
⁴⁵ Internal Medicine Department, Hospital Center of Perpignan, Perpignan, France.
⁴⁶ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France.
⁴⁷ University Hospital Center of Lille, Hematology Institute, Lille, France.
⁴⁸ Internal Medicine Department, University Hospital Center la Milétrie, CICP-1042, Poitiers, France.
⁴⁹ Nephrology and Internal Medicine Department, Hospital Center of Valenciennes, Valenciennes, France.
⁵⁰ Rheumatology Department, University Hospital Center L'Archet, Nice, France.
⁵¹ Department of Hematology, Institut Paoli-Calmettes, Marseille, France.
⁵² Hematology Department, Versailles André Mignot Hospital, University Paris-Saclay, Le Chesnay, France.
⁵³ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; University Hospital Center of Lille, Dermatology Department, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France.
⁵⁴ Pneumology Department and Referal Center for Rare Pulmonary Diseases, Bichat Hospital, Assistance Publique Hôpitaux de Paris, UMR 1152, Paris Cité University, Paris, France.
⁵⁵ Internal Medicine Department, University Hospital Center of Clermont Ferrand, Gabriel Montpied Hospital, INSERM U1071, INRA USC2018, M2iSH, Clermont Auvergne University, Clermont Ferrand, France.
⁵⁶ Internal Medicine Department, University Hospital Center of Bordeaux, Bordeaux, France.
⁵⁷ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Internal Medicine Department, Ambroise Paré Hospital, AP-HP. 9, Boulogne, France; Infection and Inflammation, UMR 1173, INSERM, UVSQ/Paris Saclay University, Montigny-le-Bretonneux, France.
⁵⁸ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Immunology Institute, Internal Medicine and Clinical Immunology Department, University Hospital Center of Lille, INFINITE-Institute for Translational Research in Inflammation, University of Lille, Lille, France; University Lille, CHU Lille, INSERM, U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France.
⁵⁹ National Referral Center for Hypereosinophilic Syndromes (CEREO), Suresnes, France; Department of Internal Medicine, Foch Hospital, Suresnes, France; University Lille, CHU Lille, INSERM, U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France. Electronic address: m.groh@hopital-foch.com.

PMID: 40992688
DOI: 10.1016/j.jaip.2025.09.009

Abstract

Background: The FIP1L1-PDFGRA (F/P)-associated hypereosinophilic syndrome (HES) is a rare condition. The 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 factor interacting with PAPOLA and CPSF1 (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 less than .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 younger than 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 8 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.

Conclusions: 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: Clinical medicine; Cost savings; Diagnosis; Diagnostic test; Eosinophilia; FIP1L1-PDGFRA fusion protein; Hypereosinophilic syndrome; Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions.

PubMed Disclaimer

LinkOut - more resources

Full Text Sources
- ClinicalKey
- Elsevier Science
Miscellaneous
- NCI CPTAC Assay Portal

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Development and Validation of the FIP Score for the Screening of FIP1L1::PDGFRA-Associated Hypereosinophilic Syndrome

Collaborators

Affiliations

Development and Validation of the FIP Score for the Screening of FIP1L1::PDGFRA-Associated Hypereosinophilic Syndrome

Authors

Collaborators

Affiliations

Abstract

LinkOut - more resources

Full Text Sources

Miscellaneous