{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T03:56:52Z","timestamp":1788753412052,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,3,30]],"date-time":"2024-03-30T00:00:00Z","timestamp":1711756800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2024,7]]},"abstract":"Abstract<\/jats:title>\n \n Background and Aims<\/jats:title>\n Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n Using the database of the French Reference Centre for Mastocytosis, we retrospectively identified patients with a liver biopsy (LB) and a diagnosis of SM. All LB procedures were performed according to the local physician in charge and centrally reviewed by an expert pathologist.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n A total of 28 patients were included: 6 had indolent SM, 9 had aggressive SM, and 13 had SM with an associated hematologic neoplasm. Twenty\u2010five (89%) patients presented hepatomegaly, and 19 (68%) had portal hypertension. The LB frequently showed slight sinusoid dilatation (82%). Fibrosis was observed in 3\/6 indolent SM and in almost all advanced SM cases (21\/22), but none of them showed cirrhosis. A high MC burden (>50 MCs\/high\u2010power field) was correlated with elevated blood alkaline phosphatase levels (\n p<\/jats:italic>\n \u2009=\u2009.030). The presence of portal hypertension was associated with a higher mean fibrosis grade (1.6 vs. 0.8 in its absence;\n p<\/jats:italic>\n \u2009=\u2009.026). In advanced SM, the presence of nodular regenerative hyperplasia (NRH) was associated with decreased overall survival (9.5 vs. 46.3\u2009months,\n p<\/jats:italic>\n \u2009=\u2009.002).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n MC infiltration induced polymorphic hepatic lesions and the degree of fibrosis is associated with portal hypertension. NRH identifies a poor prognosis subgroup of patients with advanced SM. Assessing liver histology can aid in SM prognostic evaluation.<\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/liv.15913","type":"journal-article","created":{"date-parts":[[2024,3,30]],"date-time":"2024-03-30T07:10:09Z","timestamp":1711782609000},"page":"1680-1688","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Histological characterization of liver involvement in systemic mastocytosis"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3052-1424","authenticated-orcid":false,"given":"Julien","family":"Rossignol","sequence":"first","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Canioni","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Pathology Necker Children's Hospital, AP\u2010HP, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Achille","family":"Aouba","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Normandy University School of Medicine Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Bulai\u2010Livideanu","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Dermatology H\u00f4pital Larrey, CHU Toulouse Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Barete","sequence":"additional","affiliation":[{"name":"CEREMAST, Dermatology Department Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Lancesseur","sequence":"additional","affiliation":[{"name":"CEREMAST Hematology Institute, Normandy University School of Medicine Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Polivka","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marine","family":"Madrange","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Ballul","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Neuraz","sequence":"additional","affiliation":[{"name":"Department of Bioinformatics Necker Children's Hospital, AP\u2010HP, Paris Centre University, Imagine Institute, INSERM U1163 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celine","family":"Greco","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Agopian","sequence":"additional","affiliation":[{"name":"Centre de Recherche en Canc\u00e9rologie de Marseille INSERM U1068 Marseille France"},{"name":"Association Fran\u00e7aise pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM) Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabienne","family":"Brenet","sequence":"additional","affiliation":[{"name":"Centre de Recherche en Canc\u00e9rologie de Marseille INSERM U1068 Marseille France"},{"name":"Association Fran\u00e7aise pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM) Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrice","family":"Dubreuil","sequence":"additional","affiliation":[{"name":"Centre de Recherche en Canc\u00e9rologie de Marseille INSERM U1068 Marseille France"},{"name":"Association Fran\u00e7aise pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM) Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5767-1628","authenticated-orcid":false,"given":"Richard","family":"Lemal","sequence":"additional","affiliation":[{"name":"Histocompatibility Laboratory EA 7453\u2014Universit\u00e9 Clermont Auvergne, CHU de Clermont\u2010Ferrand Clermont\u2010Ferrand France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Tournilhac","sequence":"additional","affiliation":[{"name":"CEREMAST, Adult Clinical Hematology CHU Clermont\u2010Ferrand, INSERM CIC501, EA 7453 \u2013 Clermont Auvergne University Clermont\u2010Ferrand France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Terriou","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Internal Medicine and Clinical Immunology CHU Lille Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Launay","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Internal Medicine and Clinical Immunology CHU Lille Lille France"},{"name":"Lille University, INSERM U1286 INFINITE, CHU Lille Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"Bouillet","sequence":"additional","affiliation":[{"name":"CEREMAST, Clinical Immunology\/Internal Medicine Department, National Reference Center for Angioedema Grenoble University Hospital Grenoble 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Gousseff","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Centre Hospitalier Bretagne Atlantique Vannes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edwige","family":"Le\u00a0Mouel","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Internal Medicine and Clinical Immunology Rennes University Hospital Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Hamidou","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Internal Medicine H\u00f4tel\u2010Dieu University Hospital Nantes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Neel","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Internal Medicine H\u00f4tel\u2010Dieu University Hospital Nantes 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University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Bruneau","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Pathology Necker Children's Hospital, AP\u2010HP, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludovic","family":"Lhermitte","sequence":"additional","affiliation":[{"name":"CEREMAST, Laboratory of Onco\u2010Hematology Necker Children's Hospital, APHP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Garcelon","sequence":"additional","affiliation":[{"name":"Paris Centre University, Imagine Institute, Data Science Platform, INSERM UMR 1163 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rose\u2010Marie","family":"Javier","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Rheumatology Strasbourg University Hospital Strasbourg 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R\u00e9union France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Zunic","sequence":"additional","affiliation":[{"name":"Department of Haematology Sud Reunion University Hospital Saint Pierre La R\u00e9union France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie Pierre","family":"Gourin","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Hematology CHU Dupuytren Limoges France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewa","family":"Wierzbicka\u2010Hainaut","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Dermatology CHU de Poitiers Poitiers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Fran\u00e7ois","family":"Viallard","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Infectious Diseases Haut\u2010L\u00e9v\u00eaque Hospital, CHRU Bordeaux, Bordeaux University Bordeaux 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Pierre\u2010B\u00e9nite France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Dimicoli\u2010Salazar","sequence":"additional","affiliation":[{"name":"Department of Hematology CHU de Bordeaux Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose M","family":"Torregrosa\u2010Diaz","sequence":"additional","affiliation":[{"name":"Department of Hematology CHU de Poitiers Poitiers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ang\u00e8le","family":"Soria","sequence":"additional","affiliation":[{"name":"CEREMAST, Department of Dermatology and Allergy, Tenon Hospital Sorbonne University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Arock","sequence":"additional","affiliation":[{"name":"CEREMAST, Laboratory of Hematology Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, AP\u2010HP Paris 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m\u00e9dico\u2010chirurgicales, Service d'H\u00e9patologie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2219-9201","authenticated-orcid":false,"given":"Vincent","family":"Mallet","sequence":"additional","affiliation":[{"name":"AP\u2010HP.Centre Universit\u00e9 Paris Centre Groupe Hospitalier Cochin Port Royal, DMU Canc\u00e9rologie et sp\u00e9cialit\u00e9s m\u00e9dico\u2010chirurgicales, Service d'H\u00e9patologie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Hermine","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163, AP\u2010HP, Necker Children's Hospital, Paris Centre University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghandi","family":"Damaj","sequence":"additional","affiliation":[{"name":"CEREMAST Hematology Institute, Normandy University School of Medicine Caen 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General aspects and histopathological diagnosis","volume":"12","author":"Horny HP","year":"1997","journal-title":"Histol Histopathol"},{"key":"e_1_2_15_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(86)90622-4"},{"key":"e_1_2_15_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840220423"},{"key":"e_1_2_15_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19890201)63:3<532::AID-CNCR2820630322>3.0.CO;2-F"},{"key":"e_1_2_15_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840200104"},{"key":"e_1_2_15_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-03-637728"},{"key":"e_1_2_15_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2016.190"},{"key":"e_1_2_15_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-021-01539-8"},{"key":"e_1_2_15_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1513098"},{"key":"e_1_2_15_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2022008038"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.15913","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,18]],"date-time":"2024-06-18T00:29:15Z","timestamp":1718670555000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.15913"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,30]]},"references-count":18,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2024,7]]}},"alternative-id":["10.1111\/liv.15913"],"URL":"https:\/\/doi.org\/10.1111\/liv.15913","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,30]]},"assertion":[{"value":"2023-12-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-14","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-30","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}