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Mastocytosis among elderly patients: A multicenter retrospective French study on 53 patients
Audrey Rouet1, Achille Aouba, Gandhi Damaj
1Service de médecine interne, Hôpital Tenon, Université Pierre et Marie Curie, Paris, France Service de Médecine Interne, CHU de Caen, Université Basse Normandie, Caen, France Institut d'Hématologie de Basse Normandie, Centre Hospitalier Universitaire, Université de Caen-Basse Normandie, Caen, France Faculté de Médecine et AP-HP Necker-Enfants Malades, Centre de Référence des Mastocytoses, Paris Cedex 15, France Inserm, U1068, CRCM [Signaling, Hematopoiesis and Mechanism of Oncogenesis], Institut Paoli-Calmettes,Marseille, Aix-Marseille Univ, CNRS, UMR7258, Marseille, France Mastocytosis Competence Center of Midi-Pyrénées, Department of Dermatology, Toulouse University Hospital, Toulouse, France Service de Médecine Interne, Université Claude Bernard Lyon1, Groupe Hospitalier Lyon-Sud. Chemin du Grand Revoyet, Pierre Bénite, France Service de Dermatologie, Hôpital Tenon, Université Pierre et Marie Curie-Paris 6, Paris, France Service d'Hématologie Adultes, Université Paris Descartes, Paris Sorbonne Cité, Faculté de Médecine et AP-HP Necker-Enfants Malades, Institut Imagine, Université Paris Descartes, Paris Cedex 15, France Service de Médecine interne, Hôpital St Antoine, Université Pierre et Marie Curie-Paris 6, Paris, France Service de Médecine interne, Université de Rennes 1, Hôpital Sud CHU Rennes, Rennes, France Service de Médecine interne, Hôpital Pitié Salpêtrière, Université Pierre et Marie Curie-Paris 6, Paris, France Service de Médecine interne, CHU, Nantes, France Service de Médecine interne, Hôpital Européen Georges Pompidou, Université Paris 5, Paris, France Université de Lille, UFR Médecine, Lille, France; CHRU Lille, Pôle Spécialités Médicales et Gérontologie, Département de Médecine Interne et Immunologie Clinique, Lille Cedex, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), Lille Cedex, France; LIRIC UMR 995, EA2686, France Médecine interne et Maladies vasculaires. Centre de compétences Maladies rares CHU, Angers, France Laboratoire d'Hématologie, Groupe Hospitalier Pitié-Salpêtrière 83, Bd de l'Hôpital, Paris, France LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France Université Paris Descartes, Service de Maladies Infectieuses et Tropicales, Université Paris Descartes, Sorbonne, Paris 6, AP-HP, Hôpital Necker-Enfants malades, Centre d'Infectiologie Necker-Pasteur, IHU Imagine, Paris.
Abstract:
Mastocytosis is a heterogeneous group of diseases with a young median age at diagnosis. Usually indolent and self-limited in childhood, the disease can exhibit aggressive progression in mid-adulthood. Our objectives were to describe the characteristics of the disease when diagnosed among elderly patients, for which rare data are available.The French Reference Center conducted a retrospective multicenter study on 53 patients with mastocytosis >69 years of age, to describe their clinical, biological, and genetic features.The median age of our cohort of patients was 75 years. Mastocytosis variants included were cutaneous (n = 1), indolent systemic (n = 5), aggressive systemic (n = 11), associated with a hematological non-mast cell disease (n = 34), and mast cell leukemia (n = 2). Clinical manifestations were predominantly mast cell activation symptoms (75.5%), poor performance status (50.9%), hepatosplenomegaly (50.9%), skin involvement (49.1%), osteoporosis (47.2%), and portal hypertension and ascites (26.4%). The main biological features were anemia (79.2%), thrombocytopenia (50.9%), leucopenia (20.8%), and liver enzyme abnormalities (32.1%). Of the 40 patients tested, 34 (85%), 2 (5%), and 4 (10%) exhibited the KIT D816V mutant, other KIT mutations and the wild-type form of the KIT gene, respectively. Additional sequencing detected significant genetic defects in 17 of 26 (65.3%) of the patients with associated hematological non-mast cell disease, including TET2, SRSF2, IDH2, and ASLX1 mutations. Death occurred in 19 (35.8%) patients, within a median delay of 9 months, despite the different treatment options available.Mastocytosis among elderly patients has a challenging early detection, rare skin involvement, and/or limited skin disease; it is heterogeneous and has often an aggressive presentation with nonfortuitous associated myeloid lineage malignant clones, and thus a poor overall prognosis.
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