Yannick Degboé1, Marine Eischen2, Delphine Nigon3, Pol-André Apoil4, Claire Mailhol5, Emilie Tournier6, Camille Laurent7, Katia Hanssens8, Olivier Hermine9, Carle Paul10, Michel Laroche11, Cristina Bulai-Livideanu12. 1. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France. Electronic address: degboe.y@chu-toulouse.fr. 2. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France. 3. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France. Electronic address: nigon.d@chu-toulouse.fr. 4. Department of Immunology, Rangueil Hospital, Toulouse University Hospital, Toulouse, France. Electronic address: apoil.p@chu-toulouse.fr. 5. Department of Pneumo-allergology, Larrey Hospital, Toulouse University Hospital, Toulouse, France. Electronic address: mailhol.c@chu-toulouse.fr. 6. Pathology Department, Institut Universitaire du Cancer de Toulouse - Oncopole, Toulouse, France. Electronic address: tournier.e@chu-toulouse.fr. 7. Pathology Department, Institut Universitaire du Cancer de Toulouse - Oncopole, Toulouse, France. Electronic address: laurent.c@chu-toulouse.fr. 8. Centre de Recherche en Cancérologie de Marseille, INSERM U1068, Aix-Marseille Université UM 105, CNRS UMR7258, Institut Paoli-Calmettes, Marseille, France. Electronic address: katia.hanssens@afirmm.com. 9. Department of Hematology, Université Paris Descartes, Hôpital Necker Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France. 10. Department of Dermatology, Mastocytosis Expert Center of Midi-Pyrénées, Paul Sabatier University, Toulouse University Hospital, Toulouse, France. Electronic address: paul.c@chu-toulouse.fr. 11. Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital and Paul Sabatier University, Toulouse, France. Electronic address: laroche.m@chu-toulouse.fr. 12. Department of Dermatology, Mastocytosis Expert Center of Midi-Pyrénées, Paul Sabatier University, Toulouse University Hospital, Toulouse, France. Electronic address: livideanu.c@chu-toulouse.fr.
Abstract
OBJECTIVES: Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Secondary objectives were to evaluate the ability of bone marrow tryptase (BMT) level to identify patients with FF, and to describe bone involvement in SM. METHODS: We analyzed retrospectively all consecutive patients seen in our expert center, with a diagnosis of SM according to the 2001 WHO criteria, and with complete bone assessment. We collected data about lifetime fractures, types of cutaneous manifestations, degranulation symptoms, blood and BMT levels, bone mineral density assessed by densitometry and KIT mutation. We performed a univariate analysis investigating the factors associated with FF and then a logistic multivariable regression analysis. We assessed the ability of bone marrow tryptase to identify patients with FF. RESULTS: Eighty-nine patients with SM were included. Thirty-six patients (40.4%) suffered from osteoporosis and twenty-five (28.1%) experienced lifetime FF. Univariate analysis identified age at diagnosis and disease onset, presence of telangiectasia macularis eruptiva perstans, digestive symptoms, mast cells activation symptoms, elevated BMT, low femoral and lumbar BMD, as associated with FF. Multivariate analysis identified elevated BMT, low femoral T score and older age at diagnosis as independently associated with FF. CONCLUSIONS: Low femoral T-score, BMT level, and older age at diagnosis are markers associated with FF in SM. BMT may represent an important biomarker to predict FF in SM patients.
OBJECTIVES:Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Secondary objectives were to evaluate the ability of bone marrow tryptase (BMT) level to identify patients with FF, and to describe bone involvement in SM. METHODS: We analyzed retrospectively all consecutive patients seen in our expert center, with a diagnosis of SM according to the 2001 WHO criteria, and with complete bone assessment. We collected data about lifetime fractures, types of cutaneous manifestations, degranulation symptoms, blood and BMT levels, bone mineral density assessed by densitometry and KIT mutation. We performed a univariate analysis investigating the factors associated with FF and then a logistic multivariable regression analysis. We assessed the ability of bone marrow tryptase to identify patients with FF. RESULTS: Eighty-nine patients with SM were included. Thirty-six patients (40.4%) suffered from osteoporosis and twenty-five (28.1%) experienced lifetime FF. Univariate analysis identified age at diagnosis and disease onset, presence of telangiectasia macularis eruptiva perstans, digestive symptoms, mast cells activation symptoms, elevated BMT, low femoral and lumbar BMD, as associated with FF. Multivariate analysis identified elevated BMT, low femoral T score and older age at diagnosis as independently associated with FF. CONCLUSIONS: Low femoral T-score, BMT level, and older age at diagnosis are markers associated with FF in SM. BMT may represent an important biomarker to predict FF in SM patients.
Authors: Peter Valent; Cem Akin; Karin Hartmann; Ivan Alvarez-Twose; Knut Brockow; Olivier Hermine; Marek Niedoszytko; Juliana Schwaab; Jonathan J Lyons; Melody C Carter; Hanneke Oude Elberink; Joseph H Butterfield; Tracy I George; Georg Greiner; Celalettin Ustun; Patrizia Bonadonna; Karl Sotlar; Gunnar Nilsson; Mohamad Jawhar; Frank Siebenhaar; Sigurd Broesby-Olsen; Selim Yavuz; Roberta Zanotti; Magdalena Lange; Boguslaw Nedoszytko; Gregor Hoermann; Mariana Castells; Deepti H Radia; Javier I Muñoz-Gonzalez; Wolfgang R Sperr; Massimo Triggiani; Hanneke C Kluin-Nelemans; Stephen J Galli; Lawrence B Schwartz; Andreas Reiter; Alberto Orfao; Jason Gotlib; Michel Arock; Hans-Peter Horny; Dean D Metcalfe Journal: Hemasphere Date: 2021-10-13