Histological characterization of liver involvement in systemic mastocytosis.
Julien Rossignol
(1, 2)
,
Danielle Canioni
(3, 4)
,
Achille Aouba
(5)
,
Cristina Bulai-Livideanu
(6)
,
Stéphane Barete
(7)
,
Charles Lancesseur
(5)
,
Laura Polivka
(1, 2)
,
Marine Madrange
(1, 2)
,
Thomas Ballul
(1, 2)
,
Antoine Neuraz
(1, 2)
,
Celine Greco
(1, 2)
,
Julie Agopian
(8)
,
Fabienne Brenet
(8)
,
Patrice Dubreuil
(8)
,
Richard Lemal
(9, 10, 11)
,
Olivier Tournilhac
(9, 10, 11, 12)
,
Louis Terriou
(13)
,
David Launay
(13, 14)
,
Laurence Bouillet
(15)
,
Clément Gourguechon
(16)
,
Laurent Frenzel
(1, 2)
,
Cécile Meni
(1, 2)
,
Caroline Gaudy-Marqueste
(17, 18)
,
Marie Gousseff
(19)
,
Edwige Le Mouel
(20)
,
Mohamed Hamidou
(21)
,
Antoine Neel
(21)
,
Dana Ranta
(22)
,
Roland Jaussaud
(22)
,
Philippe Guilpain
(23, 24)
,
Thierry J. Molina
(2)
,
Julie Bruneau
(2)
,
Ludovic Lhermitte
(2)
,
Nicolas Garcelon
(1)
,
Rose-Marie Javier
(25)
,
Fabien Pelletier
(26)
,
Florence Castelain
(26)
,
Frederique Retornaz
(27)
,
Quentin Cabrera
(28)
,
Patricia Zunic
(28)
,
Marie Pierre Gourin
(29)
,
Ewa Wierzbicka-Hainaut
(30)
,
Jean François Viallard
(31, 32)
,
Christian Lavigne
(33)
,
Cyrille Hoarau
(34)
,
Isabelle Durieu
(35)
,
Maël Heiblig
(36)
,
Sophie Dimicoli-Salazar
(37)
,
Jose M. Torregrosa-Diaz
(30)
,
Angèle Soria
(38, 39)
,
Michel Arock
(7)
,
Olivier Lortholary
(1, 2)
,
Christine Bodemer
(1, 2)
,
Stanislas Pol
(40)
,
Vincent Mallet
(40)
,
Olivier Hermine
(1, 2)
,
Ghandi Damaj
(41)
1
Imagine - U1163 -
Imagine - Institut des maladies génétiques (IHU)
2 Hôpital Necker - Enfants Malades [AP-HP]
3 Service de pathologie [CHU Necker]
4 UPCité - Université Paris Cité
5 UNICAEN Santé - Université de Caen Normandie - UFR Santé
6 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
7 CHU Pitié-Salpêtrière [AP-HP]
8 CRCM - Centre de Recherche en Cancérologie de Marseille
9 UCA - Université Clermont Auvergne
10 CHELTER - Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias
11 CHU Clermont-Ferrand
12 CIC 1405 - Centre d'Investigation Clinique [CHU Clermont-Ferrand]
13 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
14 INFINITE - Institute for Translational Research in Inflammation - U 1286
15 UGA UFRM - Université Grenoble Alpes - UFR Médecine
16 CHU Amiens-Picardie
17 AMU - Aix Marseille Université
18 TIMONE - Hôpital de la Timone [CHU - APHM]
19 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
20 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
21 Hôtel-Dieu de Nantes
22 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
23 Hôpital Saint Eloi [CHU Montpellier]
24 UM - Université de Montpellier
25 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
26 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
27 Hôpital Européen [Fondation Ambroise Paré - Marseille]
28 CHU Sud Saint Pierre [Ile de la Réunion]
29 Hôpital Dupuytren [CHU Limoges]
30 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
31 UB - Université de Bordeaux
32 Hôpital Haut-Lévêque [CHU Bordeaux]
33 CHU Angers - Centre Hospitalier Universitaire d'Angers
34 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
35 HCL - Hospices Civils de Lyon
36 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
37 CHU Bordeaux
38 SU - Sorbonne Université
39 CHU Tenon [AP-HP]
40 Hôpital Cochin [AP-HP]
41 UNICAEN - Université de Caen Normandie
2 Hôpital Necker - Enfants Malades [AP-HP]
3 Service de pathologie [CHU Necker]
4 UPCité - Université Paris Cité
5 UNICAEN Santé - Université de Caen Normandie - UFR Santé
6 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
7 CHU Pitié-Salpêtrière [AP-HP]
8 CRCM - Centre de Recherche en Cancérologie de Marseille
9 UCA - Université Clermont Auvergne
10 CHELTER - Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias
11 CHU Clermont-Ferrand
12 CIC 1405 - Centre d'Investigation Clinique [CHU Clermont-Ferrand]
13 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
14 INFINITE - Institute for Translational Research in Inflammation - U 1286
15 UGA UFRM - Université Grenoble Alpes - UFR Médecine
16 CHU Amiens-Picardie
17 AMU - Aix Marseille Université
18 TIMONE - Hôpital de la Timone [CHU - APHM]
19 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
20 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
21 Hôtel-Dieu de Nantes
22 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
23 Hôpital Saint Eloi [CHU Montpellier]
24 UM - Université de Montpellier
25 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
26 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
27 Hôpital Européen [Fondation Ambroise Paré - Marseille]
28 CHU Sud Saint Pierre [Ile de la Réunion]
29 Hôpital Dupuytren [CHU Limoges]
30 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
31 UB - Université de Bordeaux
32 Hôpital Haut-Lévêque [CHU Bordeaux]
33 CHU Angers - Centre Hospitalier Universitaire d'Angers
34 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
35 HCL - Hospices Civils de Lyon
36 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
37 CHU Bordeaux
38 SU - Sorbonne Université
39 CHU Tenon [AP-HP]
40 Hôpital Cochin [AP-HP]
41 UNICAEN - Université de Caen Normandie
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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