Hydroxychloroquine in Isolated Cutaneous Mastocytosis Patients or Indolent Systemic Mastocytosis With Associated Skin Involvement Patients

NCT ID: NCT05084872

Last Updated: 2021-10-20

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Clinical Phase

PHASE2/PHASE3

Total Enrollment

30 participants

Study Classification

INTERVENTIONAL

Study Start Date

2021-10-31

Study Completion Date

2024-01-31

Brief Summary

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The treatment of systemic mastocytosis has two main axes:

* Control of mast cell activation symptoms and
* The control of proliferation (accumulation) of mast cells.

There is no standard treatment and no treatment has a marketing authorization for the treatment of monoclonal indolent mastocytosis.

Detailed Description

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Mastocytosis is an orphan disease related to the accumulation and / or the proliferation of abnormal mast cells in different tissues.

In adults, a classic distinction is made between isolated cutaneous forms (10 to 15%) and systemic forms (85 to 90%).

The treatment of systemic mastocytosis has two main axes:

* Control of mast cell activation symptoms and
* The control of proliferation (accumulation) of mast cells.

There is no standard treatment and no treatment has a marketing authorization for the treatment of monoclonal indolent mastocytosis.

Conditions

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Mastocytosis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Treatment

Patients will be treated by hydroxychloroquine at a dose of 6 to 6.5mg/kg/day

Group Type EXPERIMENTAL

Hydroxychloroquine

Intervention Type DRUG

Patients will be treated by hydroxychloroquine at a dose of 6 to 6.5mg/kg/day during 12 month

Interventions

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Hydroxychloroquine

Patients will be treated by hydroxychloroquine at a dose of 6 to 6.5mg/kg/day during 12 month

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

1. Age \> 18 years
2. Isolated Cutaneous mastocytosis or indolent systemic mastocytosis with associated skin lesions defined according to WHO criteria (and / or international standards for cutaneous mastocytosis)
3. Patient with at least one disability defined by the presence of the following symptoms assessed as moderate to severe:

1. Cutaneous pruritus with score ≥ 5 on a VAS scale from 0 to 10
2. Number of flushes / week ≥ 7
4. Skin KIT mutation known
5. Performance scale: OMS/ECOG ≤ 1
6. Woman and man of childbearing age\* under effective contraception during all the treatment by hydroxychloroquine, until 8 months after its cessation

Exclusion Criteria

* Non-symptomatic mastocytosis and / or without skin involvement
* Advanced Systemic mastocytosis
* History of ophthalmic disease and / or cardiac conduction disorders, in particular the prolongation of the QT interval as well as the risk factors for prolongation of the QT interval, such as heart disease (heart failure, myocardial infarction), pro-arrhythmic conditions (eg bradycardia \<50 bpm), history of ventricular dysrhythmias, uncorrected hypokalemia and / or hypomagnesemia, concomitant treatment with interval prolonging agents QTagainst-indicating the use of hydroxychloroquine
* Treatment with citalopram, escitalopram, hydroxyzine, domperidone, piperaquine due to the increased risk of ventricular rhythm disorders, especially torsades de pointes
* Specific anti-tumor treatment (chemotherapy, radiotherapy) of less than 4 weeks before inclusion.
* Concomitant specific anti-mast cell treatment
* Contre-indication(s) to XYLOCAINE 10 mg/ml ADRENALINE 0,005 mg/ml, injectable solution: Known hypersensitivity to chlorhydrate de lidocaïne, to amide-type local anaesthetics or one of its excipients (sulfites), patients suffering from recurring porphyrias, coronary insufficiency, ventricular rhythm disorders, severe arterial hypertension, obstructive cardiomyopathy, hyperthyroidism.
* Inclusion in another trial with an experimental therapeutic molecule
* Change symptomatic treatment (including dosage) in the 4 weeks preceding the inclusion visit
* Moderate to severe renal or hepatic failure or diabetes
* History of organ transplant
* Inability to give informed consent
* Inability to undergo medical monitoring for geographical, social or psychic
* Patients with major surgery scheduled in the next two weeks screening
* Patient without health insurance
* Pregnancy, Breastfeeding
* Vulnerable Patient, defined as:
* Esperanzae survival \< 6 months
* Patient with another uncontrolled severe disease
* Patient under juridical protection
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University Hospital, Toulouse

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Maella Severino-Freire, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital, Toulouse

Locations

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Larrey Hospital - Toulouse University Hospital

Toulouse, , France

Site Status

Countries

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France

Central Contacts

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Maella Severino-Freire, MD

Role: CONTACT

05 67 77 81 41 ext. +33

Cristina Bulai Livideanu, MD

Role: CONTACT

05 67 77 81 35 ext. +33

References

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Fradet M, Negretto M, Tournier E, Laurent C, Apoil PA, Evrard S, Degboe Y, Del Mas V, Lamant L, Dubreuil P, Laroche M, Mailhol C, Hermine O, Paul C, Bulai Livideanu C. Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study. J Eur Acad Dermatol Venereol. 2019 Sep;33(9):1713-1718. doi: 10.1111/jdv.15638. Epub 2019 May 17.

Reference Type BACKGROUND
PMID: 31009132 (View on PubMed)

Severino M, Chandesris MO, Barete S, Tournier E, Sans B, Laurent C, Apoil PA, Lamant L, Mailhol C, Laroche M, Fraitag S, Hanssens K, Dubreuil P, Hermine O, Paul C, Bulai Livideanu C. Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement. J Am Acad Dermatol. 2016 May;74(5):885-91.e1. doi: 10.1016/j.jaad.2015.10.050. Epub 2016 Feb 19.

Reference Type BACKGROUND
PMID: 26899198 (View on PubMed)

Other Identifiers

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2020-003268-25

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

RC31/19/0504

Identifier Type: -

Identifier Source: org_study_id