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.
Pr Olivier Hermine
Pr Olivier Lortholary
Pr Christine Bodemer
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
- 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
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
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
- 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
DIU dermatological manifestations of systemic diseases
Paris-Est Créteil University
DU systemic diseases
Pediatric mastocytosis: long term follow up of 53 patients with whole sequencing of KIT. A prospective study.
Méni C, Georgin-Lavialle S, Le Saché de Peufeilhoux L, Jais JP, Hadj Rabia S, Bruneau J, Fraitag S, Hanssens K, Dubreuil P, Hermine O, Bodemer C. Br J Dermatol. 2018 May 22.
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
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;21
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
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
The inflammatory track to explain certain depressions
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
Le Figaro Santé | 3.29.2016
A rare disease sheds light on the mechanisms of depression
Half of patients with mastocytosis are prone to depressive disorders. Inflammatory phenomena would be involved. Read more in French
Le Monde Science et Techno | 02.29.2016
When inflammation leads to depression
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
Le Point | 2.23.2016
In Necker, the reference center for mastocytoses (CEREMAST) in brief …
* data valid for 2019