Bezuclastinib for Systemic Mastocytosis

Not currently recruiting at 77 trial locations
HJ
Overseen ByHina Jolin, PharmD
Age: 18+
Sex: Any
Trial Phase: Phase 2
Sponsor: Cogent Biosciences, Inc.
Must be taking: Antimediator therapies
Prior Safety DataThis treatment has passed at least one previous human trial

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial investigates a new treatment called bezuclastinib for individuals with nonadvanced systemic mastocytosis (NonAdvSM), a condition characterized by an excess of mast cells in the body, leading to symptoms like itching, flushing, and abdominal pain. The study aims to determine if bezuclastinib, combined with standard care, can better control these symptoms compared to a placebo (a pill with no active medicine). Participants will be randomly assigned to receive either the active treatment or a placebo, with some potentially switching to the active treatment later. This trial suits those diagnosed with indolent or smoldering systemic mastocytosis who experience moderate-to-severe symptoms not well-managed by current medications. As a Phase 2 trial, this research focuses on measuring the treatment's effectiveness in an initial, smaller group of people, offering a chance to contribute to important findings.

Do I have to stop taking my current medications for this trial?

The trial protocol does not specify if you must stop all current medications. However, you cannot take strong CYP3A4 inhibitors or inducers within 14 days before the first dose. If you're on corticosteroids, the dose must be 10 mg/day or less of prednisone or equivalent. You also need to have a stable regimen of at least 2 antimediator therapies for 14 days before joining.

Will I have to stop taking my current medications?

The trial does not specify if you must stop all current medications, but you cannot take strong CYP3A4 inhibitors or inducers within 14 days before starting the study. If you are on corticosteroids, the dose must be 10 mg/day or less of prednisone or equivalent.

Is there any evidence suggesting that this trial's treatments are likely to be safe?

Research has shown that bezuclastinib is generally safe for people with systemic mastocytosis. In earlier studies, most participants did not experience serious side effects. Some individuals reported mild to moderate issues, such as headaches or stomach upset, but these were not severe. Solid information on long-term effects is not yet available, but current evidence suggests bezuclastinib is safe for most people in the short term. As this is a Phase 2 trial, the treatment has already demonstrated some safety in earlier research, which is a positive sign.12345

Why do researchers think this study treatment might be promising for systemic mastocytosis?

Most treatments for systemic mastocytosis focus on managing symptoms rather than addressing the underlying cause. However, bezuclastinib stands out because it targets the KIT D816V mutation, which is a key driver of the disease. This specific inhibition can potentially reduce mast cell proliferation directly. Researchers are excited because this approach could offer a more effective way to control the disease progression compared to current options, which often include symptom management with antihistamines or corticosteroids.

What evidence suggests that bezuclastinib could be an effective treatment for systemic mastocytosis?

Research has shown that bezuclastinib holds promise for treating systemic mastocytosis. In studies, 94% of patients experienced at least a 50% reduction in serum tryptase, a disease marker. Additionally, 100% of patients showed a similar reduction in KIT D816V, another important marker. These decreases suggest that bezuclastinib effectively targets and reduces the activity of abnormal cells in systemic mastocytosis. In this trial, participants may receive bezuclastinib in various dosages or a placebo, alongside best supportive care (BSC). This early evidence supports the potential of bezuclastinib to improve symptoms in patients whose conditions are not well-managed with current treatments.25678

Who Is on the Research Team?

RE

Rachael Easton, MD, PhD

Principal Investigator

Cogent Biosciences

Are You a Good Fit for This Trial?

This trial is for patients with a condition called systemic mastocytosis, specifically the indolent or smoldering types. Participants should have moderate-to-severe symptoms despite current treatments and be in fairly good health overall (ECOG 0-2). They can't join if they've had significant bleeding recently, need blood thinners, have certain advanced forms of the disease, skin-only mastocytosis, previous KIT inhibitor therapy (except approved ones), recent cytoreductive therapy or radiation.

Inclusion Criteria

I am taking 10 mg or less of prednisone daily or its equivalent.
I can take care of myself and am up and about more than half of my waking hours.
I have moderate-to-severe symptoms despite taking at least 2 treatments for 14 days.
See 2 more

Exclusion Criteria

I haven't had radiotherapy or PUVA therapy in the last 14 days.
I have been treated with a KIT inhibitor, but not for systemic mastocytosis.
I have been diagnosed with mastocytosis that affects only my skin.
See 5 more

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment Part 1

Participants receive bezuclastinib or placebo to determine the recommended dose

3 months

Treatment Part 2

Participants receive bezuclastinib at the selected dose or placebo to assess efficacy and safety

24 weeks

Treatment Part 3

Participants may roll over to receive bezuclastinib for long-term efficacy and safety assessment

Up to 5 years

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

  • Bezuclastinib (CGT9486)
  • Placebo Tablets
Trial Overview The study tests bezuclastinib tablets against placebo tablets to see which is better at managing systemic mastocytosis when added to standard care. It's randomized and double-blind meaning neither doctors nor patients know who gets the real drug versus placebo. The trial has three parts; after completing Parts 1a/1b/2 with either drug or placebo, participants may move on to Part 3 for bezuclastinib treatment.
How Is the Trial Designed?
10Treatment groups
Experimental Treatment
Placebo Group
Group I: (Prior Therapy Sub-study) BezuclastinibExperimental Treatment1 Intervention
Group II: (Part 3) Bezuclastinib + BSCExperimental Treatment2 Interventions
Group III: (Part 2) Bezuclastinib Selected Dose + BSCExperimental Treatment1 Intervention
Group IV: (Part 1b) Bezuclastinib Dose 2 + BSCExperimental Treatment1 Intervention
Group V: (Part 1b) Bezuclastinib Dose 1 + BSCExperimental Treatment1 Intervention
Group VI: (Part 1a) Bezuclastinib Dose 2 + BSCExperimental Treatment1 Intervention
Group VII: (Part 1a) Bezuclastinib Dose 1 + BSCExperimental Treatment1 Intervention
Group VIII: (Part 2) Placebo + BSCPlacebo Group1 Intervention
Group IX: (Part 1a) Placebo + BSCPlacebo Group1 Intervention
Group X: (Part 1b) Placebo + BSCPlacebo Group1 Intervention

Find a Clinic Near You

Who Is Running the Clinical Trial?

Cogent Biosciences, Inc.

Lead Sponsor

Trials
11
Recruited
1,100+

Published Research Related to This Trial

A new risk score called the mutation-adjusted risk score (MARS) was developed for patients with advanced systemic mastocytosis (AdvSM), based on a study of 383 patients, which helps predict overall survival by identifying key risk factors such as age, anemia, thrombocytopenia, and specific gene mutations.
The MARS score effectively categorizes patients into low, intermediate, and high-risk groups for survival outcomes, and it is independent of the WHO classification, potentially enhancing treatment strategies for this rare condition.
MARS: Mutation-Adjusted Risk Score for Advanced Systemic Mastocytosis.Jawhar, M., Schwaab, J., Álvarez-Twose, I., et al.[2020]
In a study of 116 patients with advanced systemic mastocytosis, midostaurin demonstrated a 60% overall response rate, with 45% achieving a major response, indicating significant efficacy in treating this challenging condition.
Despite its effectiveness, midostaurin was associated with notable side effects, leading to dose reductions in 56% of patients, highlighting the need for careful monitoring and management of adverse events during treatment.
Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis.Gotlib, J., Kluin-Nelemans, HC., George, TI., et al.[2022]
Midostaurin, an oral multikinase/KIT inhibitor, has been approved for the treatment of advanced systemic mastocytosis (advSM) based on phase 2 clinical studies, showing its efficacy in managing this condition characterized by neoplastic mast cell accumulation.
Adverse events (AEs) from midostaurin can be challenging to differentiate from symptoms of the disease itself, which may lead to unnecessary treatment discontinuation; therefore, effective management strategies for AEs are crucial to ensure patients can continue benefiting from the therapy.
Practical management of adverse events in patients with advanced systemic mastocytosis receiving midostaurin.Gotlib, J., Kluin-Nelemans, HC., Akin, C., et al.[2021]

Citations

Apex Part 1: Updated Assessment of Bezuclastinib (CGT9486 ...In all Part 1 patients across dose levels, 94%, 93%, and 100% of patients achieved at least 50% reduction in serum tryptase, KIT D816V VAF, and ...
A Randomized, Double-Blind, Placebo Controlled Phase 2 ...Robust reductions in disease biomarkers were observed with bezu-100: 94.4%, 100%, and 92.3% of patients had ≥50% reduction in serum tryptase, KIT p.D816V ...
Apex Part 1: Updated Assessment of Bezuclastinib ...Herein we report updated safety and efficacy results from the fully enrolled Part 1 of the Apex trial. Methods: Apex is a randomized Phase 2 ...
Press Release Details“We are excited to share today the updated clinical data from APEX Part 1 studying bezuclastinib in patients with advanced systemic mastocytosis ...
NCT05186753 | (Summit) A Study to Evaluate the Efficacy ...This is a multi-part, randomized, double-blind, placebo-controlled Phase 2 clinical study comparing the safety and efficacy of bezuclastinib (CGT9486) plus ...
Safety and Efficacy of Bezuclastinib (CGT9486), a Novel ...Background: Systemic mastocytosis (SM) is a rare disease characterized by the aberrant proliferation of mast cells (MC).
MPN-334 Safety and Efficacy of Bezuclastinib (CGT9486) ...The primary efficacy endpoint is overall response rate (complete response [CR], CR with incomplete hematologic recovery [CRh], partial response [PR], and ...
P1049: A PHASE 2 STUDY OF BEZUCLASTINIB (CGT9486 ...Bezuclastinib, an orally administered and highly selective TKI with potent activity against KIT D816V, may be a treatment option in AdvSM.
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