Mastocytoses reference center (CEREMAST)

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.

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100 questions / réponses sur la mastocytose

Medical
team

Coordinator
Pr Olivier Hermine
MD, PhD

Olivier Lortholary

Co-coordinator
Pr Olivier Lortholary
MD, PhD

Coordinatrice : Professeur Christine Bodemer

Co-coordinator
Pr Christine Bodemer
MD, PhD

Contact us
 

Adult mastocytosis
Phone. +33 (0)1 44 49 54 16

Doctolib : prenez rendez-vous en ligne

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.

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

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

Contact information

Necker-Enfants malades university hospital
> Adult hematology department

149 rue de Sèvres
75743 PARIS Cedex 15

> Welcome booklet for hospitalized adults

> Pediatric welcome booklet

In Necker, the reference center for mastocytoses (CEREMAST) in brief …

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patients followed at least once a year*
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medical consultations*
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day hospitalizations*
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fully hospitalized patients*
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current research projects*
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university course*
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* data valid for 2019