Histological characterization of liver involvement in systemic mastocytosis.
2 Service de pathologie [CHU Necker]
3 UNICAEN Santé - Université de Caen Normandie - UFR Santé
4 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
5 CHU Pitié-Salpêtrière [AP-HP]
6 CRCM - Centre de Recherche en Cancérologie de Marseille
7 UCA - Université Clermont Auvergne
8 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
9 LIRIC - Lille Inflammation Research International Center - U 995
10 UGA UFRM - Université Grenoble Alpes - UFR Médecine
11 CHU Amiens-Picardie
12 AMU - Aix Marseille Université
13 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
14 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
15 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
16 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
17 UM - Université de Montpellier
18 Hôpital Necker - Enfants Malades [AP-HP]
19 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
20 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
21 CHU Sud Saint Pierre [Ile de la Réunion]
22 Hôpital Dupuytren [CHU Limoges]
23 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
24 UB - Université de Bordeaux
25 CHU Angers - Centre Hospitalier Universitaire d'Angers
26 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
27 HCL - Hospices Civils de Lyon
28 CHU Bordeaux - Centre Hospitalier Universitaire de Bordeaux
29 SU - Sorbonne Université
30 UNICAEN - Université de Caen Normandie
- Fonction : Auteur
- PersonId : 758051
- IdHAL : fabienne-brenet
- ORCID : 0000-0003-4098-1096
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- ORCID : 0000-0002-6475-4018
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- PersonId : 1176436
- IdHAL : christian-lavigne
- ORCID : 0000-0002-2538-0076
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- Fonction : Auteur
- PersonId : 1495108
- IdHAL : mael-heiblig
- ORCID : 0000-0003-1682-8657
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- PersonId : 1308844
- ORCID : 0000-0003-2574-3874
- IdRef : 069884927
Résumé
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.
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