Year: 2024
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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-five (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-power field) was correlated with elevated blood alkaline phosphatase levels (p = .030). The presence of portal hypertension was associated with a higher mean fibrosis grade (1.6 vs. 0.8 in its absence; p = .026). In advanced SM, the presence of nodular regenerative hyperplasia (NRH) was associated with decreased overall survival (9.5 vs. 46.3 months, p = .002). CONCLUSIONS: 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.
Source: Liver International
DOI: 10.1111/liv.15913
Authors: Julien Rossignol, Danielle Canioni, Achille Aouba, Cristina Bulai‐Livideanu, Stéphane Barete, Charles Lancesseur, Laura Polivka, Marine Madrange, Thomas Ballul, Antoine Neuraz, Celine Greco, Julie Agopian, Fabienne Brenet, Patrice Dubreuil, Richard Lemal, Olivier Tournilhac, Louis Terriou, David Launay, Laurence Bouillet, Clément Gourguechon, Laurent Frenzel, Cécile Meni, Caroline Gaudy‐Marqueste, Marie Gousseff, Edwige Le Mouel, Mohamed Hamidou, Antoine Neel, Dana Ranta, Roland Jaussaud, Philippe Guilpain, Thierry J Molina, Julie Bruneau, Ludovic Lhermitte, Nicolas Garcelon, Rose‐Marie Javier, Fabien Pelletier, Florence Castelain, Frederique Retornaz, Quentin Cabrera, Patricia Zunic, Marie Pierre Gourin, Ewa Wierzbicka‐Hainaut, Jean François Viallard, Christian Lavigne, Cyrille Hoarau, Isabelle Durieu, Maël Heiblig, Sophie Dimicoli‐Salazar, Jose M Torregrosa‐Diaz, Angèle Soria, Michel Arock, Olivier Lortholary, Christine Bodemer, Stanislas Pol, Vincent Mallet, Olivier Hermine, Ghandi Damaj, on behalf the CEREMAST Network
Institutions: Hôpital Necker-Enfants Malades, Inserm, Institut des Maladies Génétiques Imagine, Assistance Publique – Hôpitaux de Paris, Normandie Université, Université de Caen Normandie, Centre Hospitalier Universitaire de Toulouse, Hôpital Larrey, Pitié-Salpêtrière Hospital, Centre de Recherche en Cancérologie de Marseille, Université Clermont Auvergne, Centre Hospitalier Universitaire de Clermont-Ferrand, Centre Hospitalier Universitaire de Lille, Institute for Translational Research in Inflammation, Centre Hospitalier Universitaire de Grenoble, Centre Hospitalier Universitaire Amiens-Picardie, Aix-Marseille Université, Hôpital de la Timone, Centre hospitalier Bretagne Atlantique, Hôpital Pontchaillou, Centre Hospitalier Universitaire de Rennes, Centre Hospitalier Universitaire de Nantes, Centre Hospitalier Régional et Universitaire de Nancy, Université de Montpellier, Hôpital Saint Eloi, Hôpitaux Universitaires de Strasbourg, Centre Hospitalier Universitaire de Besançon, Hôpital Européen, Centre Hospitalier Universitaire de La Réunion, Hôpital Universitaire Dupuytren, Centre Hospitalier Universitaire de Poitiers, Université de Bordeaux, Centre Hospitalier Universitaire d'Angers, Centre Hospitalier Universitaire de Tours, Hospices Civils de Lyon, Hôpital Lyon Sud, Centre Hospitalier Universitaire de Bordeaux, Sorbonne Université, Hôpital Tenon, Groupe Hospitalier Cochin - Port-Royal, Hôtel-Dieu, Broca - La Collégiale
Language: English
Topic: Mast cells and histamine
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: hybrid
Estimated APC paid: $4,740
OpenAlex ID: W4393333672