Histological characterization of liver involvement in systemic mastocytosis.

Autor: Rossignol J; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Canioni D; CEREMAST, Department of Pathology, Necker Children's Hospital, AP-HP, Paris Centre University, Paris, France., Aouba A; Department of Internal Medicine, Normandy University School of Medicine, Caen, France., Bulai-Livideanu C; CEREMAST, Department of Dermatology, Hôpital Larrey, CHU Toulouse, Toulouse, France., Barete S; CEREMAST, Dermatology Department, Pitié-Salpêtrière Hospital, AP-HP, Paris, France., Lancesseur C; CEREMAST, Hematology Institute, Normandy University School of Medicine, Caen, France., Polivka L; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Madrange M; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Ballul T; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Neuraz A; Department of Bioinformatics, Necker Children's Hospital, AP-HP, Paris Centre University, Imagine Institute, INSERM U1163, Paris, France., Greco C; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Agopian J; Centre de Recherche en Cancérologie de Marseille, INSERM U1068, Marseille, France.; Association Française pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM), Marseille, France., Brenet F; Centre de Recherche en Cancérologie de Marseille, INSERM U1068, Marseille, France.; Association Française pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM), Marseille, France., Dubreuil P; Centre de Recherche en Cancérologie de Marseille, INSERM U1068, Marseille, France.; Association Française pour les Initiatives de Recherche sur le Mastocyte et les Mastocytoses (AFIRMM), Marseille, France., Lemal R; Histocompatibility Laboratory, EA 7453-Université Clermont Auvergne, CHU de Clermont-Ferrand, Clermont-Ferrand, France., Tournilhac O; CEREMAST, Adult Clinical Hematology, CHU Clermont-Ferrand, INSERM CIC501, EA 7453 - Clermont Auvergne University, Clermont-Ferrand, France., Terriou L; CEREMAST, Department of Internal Medicine and Clinical Immunology, CHU Lille, Lille, France., Launay D; CEREMAST, Department of Internal Medicine and Clinical Immunology, CHU Lille, Lille, France.; Lille University, INSERM U1286 INFINITE, CHU Lille, Lille, France., Bouillet L; CEREMAST, Clinical Immunology/Internal Medicine Department, National Reference Center for Angioedema, Grenoble University Hospital, Grenoble, France., Gourguechon C; Department of Hematology, Amiens University Hospital, Amiens, France., Frenzel L; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Meni C; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Gaudy-Marqueste C; CEREMAST, Department of Dermatology, Aix-Marseille University, CHU Timone, Marseille, France., Gousseff M; Department of Internal Medicine, Centre Hospitalier Bretagne Atlantique, Vannes, France., Le Mouel E; CEREMAST, Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France., Hamidou M; CEREMAST, Department of Internal Medicine, Hôtel-Dieu University Hospital, Nantes, France., Neel A; CEREMAST, Department of Internal Medicine, Hôtel-Dieu University Hospital, Nantes, France., Ranta D; Department of Hematology, Nancy University Hospital, Nancy, France., Jaussaud R; Department of Internal Medicine and Clinical Immunology, Nancy University Hospital, Vandoeuvre-lès-Nancy, France., Guilpain P; CEREMAST, Department of Internal Medicine-Multi-organ Diseases, Saint-Eloi University Hospital, Montpellier University, Montpellier, France., Molina TJ; CEREMAST, Department of Pathology, Necker Children's Hospital, AP-HP, Paris Centre University, Paris, France., Bruneau J; CEREMAST, Department of Pathology, Necker Children's Hospital, AP-HP, Paris Centre University, Paris, France., Lhermitte L; CEREMAST, Laboratory of Onco-Hematology, Necker Children's Hospital, APHP, Paris, France., Garcelon N; Paris Centre University, Imagine Institute, Data Science Platform, INSERM UMR 1163, Paris, France., Javier RM; CEREMAST, Department of Rheumatology, Strasbourg University Hospital, Strasbourg, France., Pelletier F; CEREMAST, Department of Dermatology, Allergology Unit, University Hospital of Besançon, Besançon, France., Castelain F; CEREMAST, Department of Dermatology, Allergology Unit, University Hospital of Besançon, Besançon, France., Retornaz F; Unité de soins et de recherche en médecine interne et maladies infectieuses, European Hospital, Marseille, France., Cabrera Q; Department of Haematology, Sud Reunion University Hospital, Saint Pierre, La Réunion, France., Zunic P; Department of Haematology, Sud Reunion University Hospital, Saint Pierre, La Réunion, France., Gourin MP; CEREMAST, Department of Hematology, CHU Dupuytren, Limoges, France., Wierzbicka-Hainaut E; CEREMAST, Department of Dermatology, CHU de Poitiers, Poitiers, France., Viallard JF; Department of Internal Medicine and Infectious Diseases, Haut-Lévêque Hospital, CHRU Bordeaux, Bordeaux University, Bordeaux, France., Lavigne C; CEREMAST, Department of Internal Medicine and Clinical Immunology, University Hospital, Angers, France., Hoarau C; CEREMAST, Service d'Immunologie Clinique et d'Allergologie, Centre Hospitalier Régional Universitaire, Tours, France., Durieu I; CEREMAST, Department of Internal Medicine, Adult Cystic Fibrosis Care Center, Hospices Civils de Lyon, Lyon, France., Heiblig M; CEREMAST, Department of Hematology, Lyon-Sud Hospital, Hospices Civils de Lyon, Pierre-Bénite, France., Dimicoli-Salazar S; Department of Hematology, CHU de Bordeaux, Bordeaux, France., Torregrosa-Diaz JM; Department of Hematology, CHU de Poitiers, Poitiers, France., Soria A; CEREMAST, Department of Dermatology and Allergy, Tenon Hospital, Sorbonne University, Paris, France., Arock M; CEREMAST, Laboratory of Hematology, Pitié-Salpêtrière Hospital, AP-HP, Paris, France., Lortholary O; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France.; Infectious and Tropical Diseases Department, Necker-Pasteur Infectiology Center, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Bodemer C; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Pol S; AP-HP.Centre Université Paris Centre, Groupe Hospitalier Cochin Port Royal, DMU Cancérologie et spécialités médico-chirurgicales, Service d'Hépatologie, Paris, France., Mallet V; AP-HP.Centre Université Paris Centre, Groupe Hospitalier Cochin Port Royal, DMU Cancérologie et spécialités médico-chirurgicales, Service d'Hépatologie, Paris, France., Hermine O; CEREMAST, Imagine Institute, INSERM U1163, AP-HP, Necker Children's Hospital, Paris Centre University, Paris, France., Damaj G; CEREMAST, Hematology Institute, Normandy University School of Medicine, Caen, France.
Jazyk: angličtina
Zdroj: Liver international : official journal of the International Association for the Study of the Liver [Liver Int] 2024 Jul; Vol. 44 (7), pp. 1680-1688. Date of Electronic Publication: 2024 Mar 30.
DOI: 10.1111/liv.15913
Abstrakt: 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.
(© 2024 The Authors. Liver International published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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