Clinical trial • Phase II • Haematology | Rare Disease

avapritinib for Indolent systemic mastocytosis | Systemic mastocytosis

Phase II trial of avapritinib for Indolent systemic mastocytosis | Systemic mastocytosis.

Overview

Trial Therapeutic Area
Haematology | Rare Disease
Trial Disease
Indolent systemic mastocytosis | Systemic mastocytosis
Trial Stage
Phase II
Drug Modality
Small molecule
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
05-09-2024
First CTIS Authorization Date
11-10-2024

Trial design

Randomised, placebo (avapritinib placebo tablets) - placebo control arm; (investigational product: avapritinib oral film-coated tablet, sponsor code blu-285; maximum daily dose listed as 100 mg)., adaptive Phase II trial across 19 sites in Spain, France, Sweden and others.

Randomised
Yes
Comparator
Placebo (Avapritinib Placebo Tablets) - placebo control arm; (investigational product: Avapritinib oral film-coated tablet, sponsor code BLU-285; maximum daily dose listed as 100 mg).
Adaptive
True - Part 1 is a dose-finding component to determine the RP2D (dose-escalation to establish recommended Phase 2 dose).
Single Multiple Or Escalation Dose Combined
Yes
Target Sample Size
137

Eligibility

Recruits 137 Vulnerable population selected (isVulnerablePopulationSelected = true). All participants must be ≥ 18 years and "must be able to give written informed consent" (inclusion criterion). No assent procedures or minor-specific consent documents are described in the provided record..

Pregnancy Exclusion
15. Pregnant women, as documented by a β-hCG pregnancy test consistent with pregnancy obtained within 7 days before the first dose of study drug.
Vulnerable Population
Vulnerable population selected (isVulnerablePopulationSelected = true). All participants must be ≥ 18 years and "must be able to give written informed consent" (inclusion criterion). No assent procedures or minor-specific consent documents are described in the provided record.

Inclusion criteria

  • {"criterion_text":"- 1. Patients must be ≥ 18 years of age.\n- 2. Patient must have SM, confirmed by Central Pathology Review of BM biopsy, and central review of B- and C-findings by WHO diagnostic criteria.\n- 3. Patient must have moderate-to- severe symptoms based on minimum mean TSS over the 14-day eligibility screening period for assessment of TSS. Minimum TSS for eligibility is ≥ 28.\n- 4. Patient must have failed to achieve adequate symptom control for 1 or more Baseline symptoms, as determined by the Investigator, with at least 2 of the following symptomatic therapies: H1 blockers, H2 blockers, proton-pump inhibitors, leukotriene inhibitors, cromolyn sodium, corticosteroids, or omalizumab.\n- 5. The patient's symptomatic SM therapies (e.g., H1 and H2 blockers) must be stable (same dose, no new medications ≥14 days before beginning the 14-day ISM-SAF eligibility TSS assessment).\n- 6. If the patient is receiving corticosteroids, the dose must be ≤20 mg/day prednisone or equivalent, and the dose must be stable for ≥14 days before beginning the 14-day ISM-SAF eligibility TSS assessment.\n- 7. Patient must have an ECOG-PS of 0 to 2.\n- 8. Patient must be able to give written informed consent."}

Exclusion criteria

  • {"criterion_text":"- 1. Patient has been diagnosed with any of the following WHO SM subclassifications: Cutaneous mastocytosis only, SM-AHN, SSM, ASM, MCL, MC sarcoma.\n- 10. Patient has a history of a cerebrovascular accident or transient ischemic attacks within 12 months before the first dose of study drug.\n- 11. Patient has a known risk or recent history (12 months before the first dose of study drug) of ICB (eg, brain aneurysm).\n- 12. Patient has a primary brain malignancy or metastases to the brain.\n- 13. Patient has clinically significant, uncontrolled cardiovascular disease, including Grade III or Grade IV congestive heart failure greater than New York Heart Association classification II; myocardial infarction or unstable angina within the previous 6 months; clinically significant, uncontrolled arrhythmias, or uncontrolled hypertension.\n- 14. Female patients who are unwilling, if not postmenopausal or surgically sterile, to abstain from sexual intercourse or employ highly effective contraception during the study drug administration period and for at least 6 weeks after the last dose of study drug. Men who are unwilling, if not surgically sterile, to abstain from sexual intercourse or employ highly effective contraception during the study drug administration period and for at least 6 weeks after the last dose of study drug.\n- 15. Pregnant women, as documented by a β-hCG pregnancy test consistent with pregnancy obtained within 7 days before the first dose of study drug.\n- 16. Women who are breastfeeding.\n- 2. Patient has been diagnosed with another myeloproliferative disorder.\n- 3. Patient has any of the following organ damage C-findings attributable to SM: Cytopenia, Hepatomegaly with ascites and impaired liver function, Palpable splenomegaly with hypersplenism, Malabsorption with hypoalbuminemia and significant weight loss, Skeletal lesions: large osteolytic lesions with pathologic fractures, Life-threatening organ damage in other organ systems that is caused by MC infiltration in tissues.\n- 4. Patient meets any of the following laboratory criteria: Aspartate aminotransferase or alanine aminotransferase > 3.0 × ULN, Total bilirubin > 1.5 × ULN; > 3.0 × ULN if due to Gilbert's disease. (In the case of Gilbert's disease, a direct bilirubin > 2.0 × ULN is an exclusion.) Albumin < 1 × LLN, eGFR < 30 mL/min/1.73 m^2 or creatinine clearance or creatinine > 1.5 × ULN Absolute neutrophil count < 1.5 × 10^9/L, Hemoglobin < 10 g/dL, Platelet count < 100,000/μL\n- 5. Patient has received any of the following medications, therapies, or procedures in the timeframes listed: Any prior treatment with avapritinib, Any TKI, including but not limited to masitinib and midostaurin, or investigational agent for < 14 days or 5 half-lives of the drug (whichever is longer) before beginning the 14-day ISM-SAF eligibility TSS assessment, Any antineoplastic drug therapy < 28 days or 5 half-lives of the drug (whichever is longer) before beginning the 14- day ISM-SAF eligibility TSS assessment, Radiotherapy or PUVA therapy < 14 days before beginning the 14-day ISM-SAF eligibility TSS assessment, Any hematopoietic growth factor < 14 days before beginning the 14-day ISM-SAF eligibility TSS assessment, Any major surgical procedure < 14 days before starting the ISM-SAF for determination of eligibility.\n- 6. Patient requires therapy with a concomitant medication that is a strong inhibitor, strong inducer, or moderate inducer of CYP3A4 (see Appendix 9).\n- 7. Patient has a history of a malignancy that has been diagnosed or required therapy within 3 years before the first dose of study drug. Patients with a prior or concurrent malignancy whose natural history or treatment does not have the potential to interfere with the safety or efficacy assessment of the investigational agent may be included after approval by medical monitor.\n- 8. Patient has a QTcF > 480 msec.\n- 9. Patient has a history of a seizure disorder (eg, epilepsy) or requires antiseizure medication."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Part 1 •The RP2D in patients with ISM.\n- Part 2 •Mean change in ISM-SAF TSS, from Baseline to C7D1.\n- Part 3 •The long-term safety and efficacy of avapritinib.","definition_or_measurement_approach":"Part 1: determination of RP2D (recommended Phase 2 dose) in Part 1 (dose-finding cohort). Part 2: mean change in ISM-SAF TSS from Baseline to Cycle 7 Day 1 compared to placebo using the ISM-SAF instrument. Part 3: assessment of long-term safety and efficacy via safety monitoring and efficacy assessments over the long-term treatment period."}

Secondary endpoints

  • {"endpoint_text":"- • Change in measures of mast cell burden: serum tryptase, KIT D816V allele burden in blood, bone marrow mast cells.\n- • Change in best supportive care usage.\n- • Change in MC-QoL, PGIS, SF-12, PGIC, and EQ-5D-5L.\n- • Safety and tolerability of avapritinib, as assessed by AEs, vital signs, electrocardiograms, and laboratory tests.\n- • Pharmacokinetics of avapritinib (Part 1 and 2 only)","definition_or_measurement_approach":"Mast cell burden: measured by serum tryptase, KIT D816V allele fraction in peripheral blood, and bone marrow mast cell assessment. Supportive care usage captured as change in best supportive care. Patient-reported outcomes captured via MC-QoL, PGIS, SF-12, PGIC, EQ-5D-5L instruments. Safety via adverse events, vital signs, ECGs, laboratory tests. PK assessed in Parts 1 and 2."}

Recruitment

Planned Sample Size
137
Recruitment Window Months
100
Consent Approach
Participants must provide written informed consent themselves (inclusion criterion: "Patient must be able to give written informed consent"). Multiple country-specific ICFs are included in the submission (documents in English and in-country translations such as Spanish, French, Dutch, Swedish, Italian, Norwegian, German, Danish and local variants are present among submitted ICF documents). No assent procedures for minors are described; minimum age is ≥18.

Geography

Total Number Of Sites
19
Total Number Of Participants
114

Spain

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
21-04-2026
Number Of Sites
2
Number Of Participants
17

Sites

Site Name
Hospital Virgen Del Valle
Department Name
Instituto de Estudios de Mastocitosis de Castilla La Mancha
Contact Person Name
Ivan Alvarez-Twose
Contact Person Email
iavana@sescam.jccm.es
Site Name
Hospital Universitari Vall D Hebron
Department Name
Internal Medice
Contact Person Name
Mar Guilarte Clavero
Contact Person Email
mguilarte@vhebron.net

France

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
22-04-2026
Number Of Sites
3
Number Of Participants
17

Sites

Site Name
Hopitaux Universitaires Pitie Salpetriere
Department Name
Service de Dermatologie
Contact Person Name
Stephane BARETE
Contact Person Email
stephane.barete@aphp.fr
Site Name
Centre Hospitalier Universitaire De Toulouse
Department Name
Service de Dermatologie
Contact Person Name
Cristina LIVIDEANU
Contact Person Email
livideanu.c@chu-toulouse.fr
Site Name
Centre Hospitalier Regional De Marseille
Department Name
Service de Dermatologie et Cancerologie Cutanee
Contact Person Name
Caroline GAUDY-MARQUESTE
Contact Person Email
caroline.gaudy@ap-hm.fr

Sweden

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
22-04-2026
Number Of Sites
2
Number Of Participants
3

Sites

Site Name
Karolinska University Hospital
Department Name
Hematologimottagningen R51
Contact Person Name
Johanna Ungerstedt
Contact Person Email
johanna.ungerstedt@ki.se
Site Name
Uppsala University Hospital
Department Name
Hematologmottagningen/101A
Contact Person Name
Mattias Mattsson
Contact Person Email
mattias.mattsson@akademiska.se

Netherlands

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
30-04-2026
Number Of Sites
2
Number Of Participants
18

Sites

Site Name
Erasmus Universitair Medisch Centrum Rotterdam (Erasmus MC)
Department Name
Immunology
Contact Person Name
Paulus van Daele
Contact Person Email
p.l.a.vandaele@eramsusmc.nl
Site Name
Universitair Medisch Centrum Groningen
Department Name
Allergology
Contact Person Name
Hanneke Oude Elberink
Contact Person Email
j.n.g.oude.elberink@umcg.nl

Belgium

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
12-05-2026
Number Of Sites
1
Number Of Participants
10

Sites

Site Name
Antwerp University Hospital
Department Name
Department of Immunology-allergology
Contact Person Name
Vito Sabato
Contact Person Email
vito.sabato@uza.be

Denmark

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
17-04-2026
Number Of Sites
1
Number Of Participants
1

Sites

Site Name
Odense University Hospital
Department Name
ORCA/ Allergicentret Hudafdeling I og Allergicenter
Contact Person Name
Sigurd Broesby-Olsen
Contact Person Email
OUH.ODE.I.Blueprint@rsyd.dk

Norway

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
20-04-2026
Number Of Sites
1
Number Of Participants
8

Sites

Site Name
Oslo University Hospital HF
Department Name
Rikshospitalet Seksjon Klinisk Forskningspost
Contact Person Name
Ingunn Dybedal
Contact Person Email
idybedal@ous-hf.no

Germany

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
22-04-2026
Number Of Sites
5
Number Of Participants
34

Sites

Site Name
Universitaetsklinikum Schleswig-Holstein AöR
Department Name
Haematologie- Onkologie
Contact Person Name
Friederike Wortmann
Contact Person Email
friederike.wortmann@uksh.de
Site Name
Charite Universitaetsmedizin Berlin KöR
Department Name
Institut für Allergologie
Contact Person Name
Frank Siebenhaar
Contact Person Email
frank.siebenhaar@charite.de
Site Name
Universitaetsklinikum Aachen AöR
Department Name
Klinik für Haematologie Onkologie Haemostaseologie und Stammzelltransplantation
Contact Person Name
Jens Panse
Contact Person Email
jpanse@ukaachen.de
Site Name
Universitaetsklinikum Mannheim GmbH
Department Name
Medizinische Klinik III
Contact Person Name
Andreas Reiter
Site Name
University Medical Center Hamburg-Eppendorf
Department Name
Abteilung für Onkologie und Haematologie
Contact Person Name
Philippe Schafhausen
Contact Person Email
schafhausen@uke.de

Italy

Earliest CTIS Part Ii Submission Date
18-09-2024
Latest Decision Or Authorization Date
20-04-2026
Number Of Sites
2
Number Of Participants
6

Sites

Site Name
Centro Ricerche Cliniche Di Verona S.r.l.
Department Name
UOC Allergologia e asma center
Contact Person Name
Elisa Olivieri
Contact Person Email
elisa.olivieri2@aovr.veneto.it
Site Name
Azienda Ospedaliera Universitaria San Giovanni Di Dio E Ruggi d'Aragona
Department Name
Unita’ Operativa Complessa di Immunologia Clinica e Reumatologia
Contact Person Name
Massimo Triggiani
Contact Person Email
mtriggiani@unisa.it

Sponsor

Primary sponsor

Full Name
Blueprint Medicines Corp.
Organisation Type
Pharmaceutical company
Country Of Registered Address
United States

Contract research organisations

Name
Syneos Health Netherlands B.V.
Name
PPD (UK) Limited
Responsibilities
PV reporting
Name
Endpoint Clinical Inc.
Name
Biotel Research LLC
Responsibilities
Imaging services
Name
Signant Health Global LLC
Responsibilities
E-data capture - QOL questionnaire

Third parties

  • {"country":"United States","full_name":"Arup Laboratories Inc.","duties_or_roles":"Central Pathology Lab- Analysis of bone marrow samples for SM diagnosis and biomarker analysis","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"Medpace Reference Laboratories LLC","duties_or_roles":"Central lab for tryptase & sample management","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United Kingdom","full_name":"Mde Services Group Limited","duties_or_roles":"Patient Reimbursement","organisation_type":"Hospital/Clinic/Other health care facility"}
  • {"country":"United Kingdom (Northern Ireland)","full_name":"Almac Clinical Services Limited","duties_or_roles":"IMP Packaging and distribution - EU QP Release","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Almac Clinical Services (Ireland) Limited","duties_or_roles":"• IMP Packaging and distribution • EU QP Release","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Endpoint Clinical Inc.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Biotel Research LLC","duties_or_roles":"Imaging services","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Frontage Laboratories Inc.","duties_or_roles":"PK analyses","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Canfield Scientific Inc.","duties_or_roles":"Skin photography","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Molecularmd Corp.","duties_or_roles":"DNA Mutation Analysis","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Signant Health Global LLC","duties_or_roles":"E-data capture - QOL questionnaire","organisation_type":"Pharmaceutical company"}
  • {"country":"Netherlands","full_name":"Syneos Health Netherlands B.V.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"PPD (UK) Limited","duties_or_roles":"PV reporting","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
Avapritinib
Active Substance
avapritinib
Modality
Small molecule
Routes Of Administration
Oral
Route
Oral
Authorisation Status
Authorised
Orphan Designation
Yes
Maximum Dose
100 mg daily

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