French national reference center for mastocytoses (CEREMAST)
The rare diseases reference center for mastocytoses (CEREMAST) takes care of adults and children with mastocytosis, whatever the form they present (cutaneous, indolent systemic, aggressive, associated with hematological diseases) and syndromes of mast cell activation. It also aims to better understand mastocytosis and to disseminate updated information to all of our partners.
The center’s missions are to:
- allow the diagnosis of the disease and optimize the care of patients and their families;
- assist in the care of patients living in provinces by providing a link with local correspondents;
- harmonize medical practices and work with specialized care relays;
- train and inform patients and their families: (Booklet “100 questions – 100 answers);
- work with the CPAM and MDPH to recognize functional disability as a disabling polypathology;
- promote and develop clinical and fundamental research programs;
- participate in epidemiological surveillance;
- promote and develop teaching on mastocytosis.
This reference center is affiliated with the MARIH rare diseases healthcare network , the EuroBloodNet European networks (ERN) and the European Competence Network on Mastocytosis.
Medical
team
Coordinator
Pr Olivier Hermine
MD, PhD
Co-coordinator
Pr Olivier Lortholary
MD, PhD
Co-coordinator
Pr Christine Bodemer
MD, PhD
Contact us
Adult mastocytosis
Phone. +33 (0)1 44 49 54 16
Childhood mastocytosis
Phone. +33 (0)1 44 49 46 68
Phone. +33 (0)1 44 49 46 72
In case of emergency
Go to local emergencies or contact the SAMU depending on the degree of emergency
Request for an opinion
Phone. +33 (0)1 44 49 40 00 (ask for the hematologist on call)
This is a heterogeneous group of diseases characterized by an accumulation, more rarely an abnormal proliferation of mast cells, in various tissues (mainly the skin in the case of pigmentary urticaria) and their unregulated activation (degranulation).
The mast cell comes from the bone marrow but only the very rare aggressive forms progress as a disease of the bone marrow. In the majority of cases, the disorders result from degranulation and not from the accumulation of mastocytoses.
Symptoms of mast cell degranulation
Spontaneously or under the effect of certain factors (food, drugs, friction, temperature variations, etc.) activated mast cells release the contents of their granules. The released mediators (histamine, tryptase, etc.) can cause local or even remote reactions in all organs after blood circulation: skin (flushing, itching), digestive tract (pain, diarrhea), heart-vessels (malaise , palpitations), musculoskeletal sphere (pain, osteoporosis, bone lysis), others (fatigue, headaches, sleep and mood disorders, anaphylactic shock, frequent urination, cough, etc.).
Diagnostic and treatment modalities
The diagnosis can sometimes be made simply on physical examination. Additional examinations are often necessary for diagnostic, prognostic and therapeutic purposes and may include: a skin or other organ (bone marrow) biopsy, a tryptase test, a search for the c-kit mutation, a bone densitometry. In children, clinical examination is usually sufficient.
Treatment depends on the type of mastocytosis. It includes the avoidance of triggering factors, molecules blocking degranulation and / or mast cell proliferation and innovative treatments in the context of therapeutic trials.
- Cutaneous mastocytosis
- Mastocytoma
- Indolent, bordeline and aggressive systemic mastocytosis
- Systemic mastocytosis associated with non-mast cell blood disease
- Mast cell leukemia
- Mast cell sarcoma
- Mast cell activation syndrome
Ascites: accumulation of fluid in the abdomen
Asthenia: fatigue
Biopsy: study of a piece of tissue taken from a living being to establish a diagnosis
Bone densitometry: rapid, non-invasive, low-irradiation X-ray examination
Bone marrow biopsy: removal of a fragment of bone marrow tissue, the sample collected is solid
Darier’s sign: redness on rubbing of a skin lesion of mastocytosis
Dysuria: difficulty urinating
Flush: hot flash
Hymenoptera: insect
Micturition : action of urinating
Myelogram: aspiration of bone marrow, the sample collected is liquid like blood
Nocturia: getting up frequently at night to urinate
Osteoporosis: decrease in bone mass responsible for excessive skeletal fragility
Pollakiuria: excessive frequency of urination
Pruritus: itching
- Pr Olivier Hermine, haematologist – responsible for the structure
- Pr Olivier Lortholary, internist
- Pr Christine Bodemer, pediatric dermatologist
- Dr Julien Rossignol, haematologist – referring doctor of the structure
- Dr Laura Polivka, dermatologist pediatrician
- Dr Céline Greco, pain specialist
- Dr Laurent Frenzel, rheumatologist specializing in mastocytosis
- Dr Cécile Meni, dermatologist pediatrician
- Hassiba Bouktit, clinical research associate
- Anne-Florence Bellais-Collange, coordination assistant
- Catherine Reichert, clinical psychologist
- International multicentre phase III study: efficacy and safety of midostaurin in patients with aggressive systemic mastocytosis or mast cell leukemia (Nejm, 2016)
- International Multicenter Phase III Study: Masitinib Treatment for Patients with Indolent Systemic Mastocytoses with severe symptoms (Lancet, 2017)
- AFIRMM diagnostic and pathophysiological protocol (in progress)
DIU hematology national internal medicine
Paris-Est, Paris-Descartes Universities
DIU Pediatric dermatology
Web teaching
DIU dermatological manifestations of systemic diseases
Paris-Est Créteil University
DU systemic diseases
Paris-Descartes University
2021
– A New Digital Method for Counting Mast Cells in Cutaneous Specific Lesions of Mastocytosis: A Series of Adult Cases of Mastocytosis With Clinical-Pathological Correlations.
Philippe Drabent, Rana Mitri, Gilles Le Naour, Olivier Hermine, Julien Rossignol, Thierry Jo Molina, Stéphane Barete, Sylvie Fraitag
Am J Dermatopathol, 2021 Jan 1, PMID: 32568831 DOI: 10.1097/DAD.0000000000001705
– Substitution of opioids by N-methyl-D-aspartate receptor antagonists for treating pain in mastocytosis.
Julien Rossignol, Nelly Schinkel-Le Nagard, Laurent Frenzel, Olivier Hermine, Céline Greco
Ann Allergy Asthma Immunol, 2021 Mar, PMID: 33259920 DOI: 10.1016/j.anai.2020.11.013
– Criteria for the Regression of Pediatric Mastocytosis: A Long-Term Follow-Up.
Laura Polivka, Julien Rossignol, Antoine Neuraz, Djiba Condé, Julie Agopian, Cécile Méni, Nicolas Garcelon, Patrice Dubreuil, Leïla Maouche-Chrétien, Smail Hadj-Rabia, Olivier Hermine, Christine Bodemer
J Allergy Clin Immunol Pract, 2021 Apr, PMID: 33338682 DOI: 10.1016/j.jaip.2020.12.019
– The association of Greig syndrome and mastocytosis reveals the involvement of the hedgehog pathway in advanced mastocytosis.
L Polivka, V Parietti, J Bruneau, E Soucie, M Madrange, E Bayard, R Rignault, D Canioni, S Fraitag, L Lhermitte, M Feroul, M Tissandier, J Rossignol, L Frenzel, N Cagnard, C Meni, H Bouktit, A-F Collange, C Gougoula, M Parisot, B Bader-Meunier, C Livideanu, C Laurent, M Arock, S Hadj-Rabia, U Rüther, P Dubreuil, C Bodemer, O Hermine, L Maouche-Chrétien
Blood, 2021 Dec 9, PMID: 34424959 DOI: 10.1182/blood.2020010207
– Long-term survival analysis of masitinib in amyotrophic lateral sclerosis.
Jesus S Mora, Walter G Bradley, Delia Chaverri, María Hernández-Barral, Javier Mascias, Josep Gamez, Gisella M Gargiulo-Monachelli, Alain Moussy, Colin D Mansfield, Olivier Hermine, Albert C Ludolph
Ther Adv Neurol Disord, 2021 Jul 19, PMID: 34457038 PMCID: PMC8388186 DOI: 10.1177/17562864211030365
– Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin.
David Fuchs, Alex Kilbertus, Karin Kofler, Nikolas von Bubnoff, Khalid Shoumariyeh, Roberta Zanotti, Patrizia Bonadonna, Luigi Scaffidi, Michael Doubek et al.
J Allergy Clin Immunol Pract, 2021 Apr, PMID: 33346151 DOI: 10.1016/j.jaip.2020.12.022
– Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis.
Hanneke C Kluin-Nelemans, Mohamad Jawhar, Andreas Reiter, Bjorn van Anrooij, Jason Gotlib, Karin Hartmann, Anja Illerhaus, Hanneke N G Oude Elberink et al.
Theranostics, 2021 Jan 1, PMID: 33391475 PMCID: PMC7681091 DOI: 10.7150/thno.51872
– Cutaneous mastocytosis in adults with a serum tryptase level < 20 ng mL -1 : why we should investigate further.
F Jendoubi, J Shourick, M Negretto, C Laurent, P A Apoil, S Evrard, V Mansat-De Mas, M Severino-Freire, C Mailhol, Y Degboé, P Dubreuil, O Hermine, C Paul, C Bulai Livideanu
Br J Dermatol, 2021 Aug, PMID: 33811770 DOI: 10.1111/bjd.20098
– COVID-19 Vaccination in Mastocytosis: Recommendations of the European Competence Network on Mastocytosis (ECNM) and American Initiative in Mast Cell Diseases (AIM).
Patrizia Bonadonna, Knut Brockow, Marek Niedoszytko, Hanneke Oude Elberink, Cem Akin, Boguslaw Nedoszytko, Joseph H Butterfield, Ivan Alvarez-Twose, Karl Sotlar et al.
J Allergy Clin Immunol Pract, 2021 Jun, PMID: 33831618 PMCID: PMC8019658 DOI: 10.1016/j.jaip.2021.03.041
– GlcNAc is a mast-cell chromatin-remodeling oncometabolite that promotes systemic mastocytosis aggressiveness.
Julie Agopian, Quentin Da Costa, Quang Vo Nguyen, Giulia Scorrano, Paraskevi Kousteridou, Min Yuan, Rabie Chelbi, Armelle Goubard, Remy Castellano, Julien Maurizio, Cristina Teodosio, Paulo De Sepulveda, John M Asara, Alberto Orfao, Olivier Hermine, Patrice Dubreuil, Fabienne Brenet
Blood, 2021 Oct 28, PMID: 33974006 DOI: 10.1182/blood.2020008948
– Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review.
Theo Gülen, Cem Aki, Patrizia Bonadonna, Frank Siebenhaar, Sigurd Broesby-Olsen, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Hanneke N G Oude Elberink, Joseph H Butterfield et al.
J Allergy Clin Immunol Pract, 2021 Nov, PMID: 34166845 DOI: 10.1016/j.jaip.2021.06.011
2020
– Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry.
Hanneke C. Kluin-Nelemans, Andreas Reiter, Anja Illerhaus, Bjorn van Anrooij, Karin Hartmann, Lambertus F. R. Span, Aleksandra Gorska, Marek Niedoszytko, Magdalena Lange, Luigi Scaffidi, Roberta Zanotti, Patrizia Bonadonna, Cecelia Perkins, Chiara Elena, Luca Malcovati, Khalid Shoumariyeh, Nikolas von Bubnoff, Roberta Parente, Massimo Triggiani, Juliana Schwaab, Mohamad Jawhar, Francesca Caroppo, Anna Belloni Fortina, Knut Brockow, Alexander Zink, David Fuchs, Alex Kilbertus, Akif Selim Yavuz, Michael Doubek, Mattias Mattsson, Hans Hagglund, Jens Panse, Vito Sabato, Elisabeth Aberer, Dietger Niederwieser, Christine Breynaert, Judit Várkonyi, Vanessa Kennedy, Olivier Lortholary, Thilo Jakob, Olivier Hermine, Julien Rossignol, Michel Arock, Jason Gotlib, Peter Valent & Wolfgang R. Sperr
Leukemia, 2020 Apr, PMID: 31740811 PMCID: PMC7115841 DOI: 10.1038/s41375-019-0632-4
– Mastocytosis onset in a patient with treated hairy cell leukemia: Just a coincidence?
Alice Boilève, Adrien Contejean, Sylvain Barreau, Élise Sourdeau, Chloé Friedrich, Olivier Kosmider, Barbara Burroni, Nicolas Dupin, Coralie Lheure, Julien Rossignol, Didier Bouscary, Éric Grignano
Blood Cells Mol Dis, 2020 Mar, PMID: 31794934 DOI: 10.1016/j.bcmd.2019.102392
– Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.
Jakub Trizuljak,Wolfgang R. Sperr,Lucie Nekvindová,Hanneke O. Elberink,Karoline V. Gleixner,Aleksandra Gorska,Magdalena Lange,Karin Hartmann,Anja Illerhaus,Massimiliano Bonifacio,Cecelia Perkins,Chiara Elena,Luca Malcovati,Anna B. Fortina,Khalid Shoumariyeh,Mohamad Jawhar,Roberta Zanotti,Patrizia Bonadonna,Francesca Caroppo,Alexander Zink,Massimo Triggiani,Roberta Parente,Nikolas von Bubnoff,Akif S. Yavuz,Hans Hägglund,Mattias Mattsson,Jens Panse,Nadja Jäkel,Alex Kilbertus,Olivier Hermine,Michel Arock,David Fuchs,Vito Sabato,Knut Brockow,Agnes Bretterklieber,Marek Niedoszytko,Björn van Anrooij,Andreas Reiter,Jason Gotlib,Hanneke C. Kluin-Nelemans,Jiri Mayer,Michael Doubek,Peter Valent
Allergy, 2020 Aug, PMID: 32108361 PMCID: PMC7115854 DOI: 10.1111/all.14248
– Midostaurin improves quality of life and mediator-related symptoms in advanced systemic mastocytosis.
Karin Hartmann, Jason Gotlib, Cem Akin, Olivier Hermine, Farrukh T Awan, Elizabeth Hexner, Michael J Mauro, Hans D Menssen, Suman Redhu, Stefanie Knoll, Karl Sotlar, Tracy I George, Hans-Peter Horny, Peter Valent, Andreas Reiter, Hanneke C Kluin-Nelemans
J Allergy Clin Immunol, 2020 Aug, PMID: 32437738 DOI: 10.1016/j.jaci.2020.03.044
– Risk and management of patients with mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) pandemic: Expert opinions.
Peter Valent, Cem Akin, Patrizia Bonadonna, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Joseph H Butterfield, Ivan Alvarez-Twose, Karl Sotlar, Juliana Schwaab, Mohamad Jawhar, Andreas Reiter, Mariana Castells, Wolfgang R Sperr, Hanneke C Kluin-Nelemans, Olivier Hermine, Jason Gotlib, Roberta Zanotti, Sigurd Broesby-Olsen, Hans-Peter Horny, Massimo Triggiani, Frank Siebenhaar, Alberto Orfao, Dean D Metcalfe, Michel Arock, Karin Hartmann
J Allergy Clin Immunol, 2020 Aug, PMID: 32561389 PMCID: PMC7297685 DOI: 10.1016/j.jaci.2020.06.009
– Mast cells as a unique hematopoietic lineage and cell system: From Paul Ehrlich »s visions to precision medicine concepts.
Peter Valent, Cem Akin, Karin Hartmann, Gunnar Nilsson, Andreas Reiter, Olivier Hermine, Karl Sotlar, Wolfgang R Sperr, Luis Escribano, Tracy I George, Hanneke C Kluin-Nelemans, Celalettin Ustun, Massimo Triggiani, Knut Brockow, Jason Gotlib, Alberto Orfao, Petri T Kovanen, Emir Hadzijusufovic , Irina Sadovnik, Hans-Peter Horny, Michel Arock, Lawrence B Schwartz, K Frank Austen, Dean D Metcalfe, Stephen J Galli
Theranostics, 2020 Aug 29, PMID: 32929378 PMCID: PMC7482799 DOI: 10.7150/thno.46719
– Mast cells in spondyloarthritis, more than simple inflammatory bystanders?
Laura Polivka, Laurent Frenzel, Jean-Yves Jouzeau, Olivier Hermine, David Moulin
Ther Adv Musculoskelet Dis, 2020 Nov 16, PMID: 33240405 PMCID: PMC7675857 DOI: 10.1177/1759720X20971907
2018
– Pediatric mastocytosis: long term follow up of 53 patients with whole sequencing of KIT. A prospective study.
C Meni, S Georgin-Lavialle, L Le Saché de Peufeilhoux, J P Jais, S Hadj-Rabia, J Bruneau, S Fraitag, K Hanssens, P Dubreuil, O Hermine, C Bodemer
Br J Dermatol, 2018 Oct, PMID: 29787623 DOI: 10.1111/bjd.16795
2017
– Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study.
Lortholary O, Chandesris MO, Bulai Livideanu C, Paul C, Guillet G, Jassem E, Niedoszytko M, Barete S, Verstovsek S, Grattan C, Damaj G, Canioni D, Fraitag S, Lhermitte L, Georgin Lavialle S, Frenzel L, Afrin LB, Hanssens K, Agopian J, Gaillard R, Kinet JP, Auclair C, Mansfield C, Moussy A, Dubreuil P, Hermine O. Lancet. 2017 Feb 11, PMID: 28069279 PMCID: PMC5985971 DOI: 10.1016/S0140-6736(16)31403-9
2016
– Mast cells’ involvement in inflammation pathways linked to depression: evidence in mastocytosis.
Georgin-Lavialle S, Moura DS, Salvador A, Chauvet-Gelinier JC, Launay JM, Damaj G, Côté F, Soucié E, Chandesris MO, Barète S, Grandpeix-Guyodo C, Bachmeyer C, Alyanakian MA, Aouba A, Lortholary O, Dubreuil P, Teyssier JR, Trojak B, Haffen E, Vandel P, Bonin B; French Mast Cell Study Group, Hermine O, Gaillard R.
Mol Psychiatry. 2016 Nov, PMID: 26809839 DOI: 10.1038/mp.2015.216
– Midostaurin in Advanced Systemic Mastocytosis.
Chandesris MO, Damaj G, Canioni D, Brouzes C, Lhermitte L, Hanssens K, Frenzel L, Cherquaoui Z, Durieu I, Durupt S, Gyan E, Beyne-Rauzy O, Launay D, Faure C, Hamidou M, Besnard S, Diouf M, Schiffmann A, Niault M, Jeandel PY, Ranta D, Gressin R, Chantepie S, Barete S, Dubreuil P, Bourget P, Lortholary O, Hermine O; CEREMAST Study Group.
N Engl J Med. 2016 Jun 30, PMID: 27355555 DOI: 10.1056/NEJMc1515403
– Mastocytosis among elderly patients: A multicenter retrospective French study on 53 patients.
Rouet A, Aouba A, Damaj G, Soucié E, Hanssens K, Chandesris MO, Livideanu CB, Dutertre M, Durieu I, Grandpeix-Guyodo C, Barète S, Bachmeyer C, Soria A, Frenzel L, Fain O, Grosbois B, de Gennes C, Hamidou M, Arlet JB, Launay D, Lavigne C, Arock M, Lortholary O, Dubreuil P, Hermine O, Georgin-Lavialle S.
Medicine (Baltimore), 2016 Jun, PMID: 27310990 PMCID: PMC4998476 DOI: 10.1097/MD.0000000000003901
Systemic, cutaneous, digestive mastocytosis: causes, symptoms, treatments
Femme actuelle | 5.15.2022
Mastocytosis is a rare disease. It is polymorphic and is the consequence of an abnormal accumulation of mast cells (cells whose role is essential for the balance of the organism) in the skin or in an organ.
> Read more in French
The inflammatory track to explain certain depressions
Le Figaro Santé | 3.29.2016
The study of a rare disease, mastocytosis, reveals that an inflammatory mechanism can be at the origin of some resistant depressions. Hope for new treatments.
> Read more in French
A rare disease sheds light on the mechanisms of depression
Le Monde Science et Techno | 2.29.2016
Half of patients with mastocytosis are prone to depressive disorders. Inflammatory phenomena would be involved.
> Read more in French
When inflammation leads to depression
Le Point | 2.23.2016
While studying a rare disease, researchers have discovered a new mechanism that may be at the origin of this so common psychological problem.
> Read more in French
Contact information
Necker-Enfants malades university hospital
> Adult hematology department
149 rue de Sèvres
75743 PARIS Cedex 15
> Welcome booklet for hospitalized adults
In Necker, the reference center for mastocytoses (CEREMAST) in brief …
* data valid for 2022