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Study of Select Combinations in Adults With Myelofibrosis

Phase 1
Withdrawn
Conditions
Primary Myelofibrosis
Myelofibrosis
PMF
Post-Essential Thrombocythemia Myelofibrosis
Post-Polycythemia Vera Myelofibrosis
Interventions
Drug: MBG453
Drug: NIS793
Drug: Decitabine
Drug: Spartalizumab
Registration Number
NCT04283526
Lead Sponsor
Novartis Pharmaceuticals
Brief Summary

The purpose of this study is to investigate the safety, pharmacokinetics (PK) and preliminary efficacy of both the combination of MBG453 and NIS793 with or without decitabine or spartalizumab as well as single agent MBG453 and/or NIS793 single agent in myelofibrosis (MF) subjects post treatment with a Janus Kinase (JAK) inhibitor.

In this study, combination therapies with novel agents including immune therapy will focus on determining the promising combinations that provide acceptable safety and efficacy independent of JAK inhibitors. Immune therapy combinations, such as MBG453 in combination with NIS793, might offer the potential to target MF across genetic heterogeneity.

The primary objective of this study is to characterize the safety, tolerability and recomended dose for each treatment combination (MBG453 + NIS793, MBG453 + NIS793 + decitabine, and MBG453 + NIS793 + spartalizumab)

Detailed Description

The purpose of this study is to investigate the safety, pharmacokinetics (PK) and preliminary efficacy of both the combination of MBG453 and NIS793 with or without decitabine or spartalizumab as well as single agent MBG453 and/or NIS793 single agent in myelofibrosis (MF) subjects post treatment with a Janus Kinase (JAK) inhibitor.

In this study, combination therapies with novel agents including immune therapy will focus on determining the promising combinations that provide acceptable safety and efficacy independent of JAK inhibitors. Immune therapy combinations, such as MBG453 in combination with NIS793, might offer the potential to target MF across genetic heterogeneity.

The primary objective of this study is to characterize the safety, tolerability and recomended dose for each treatment combination (MBG453 + NIS793, MBG453 + NIS793 + decitabine, MBG453 + NIS793 + spartalizumab).

Secondary Objectives are: to evaluate the efficacy based on the revised International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) response criteria, to evaluate the effect of each combination treatment in delaying progression of MF and estimate time to progression free survival (PFS) event, and to characterize the PK profile of each treatment arm Study is designed as a Phase Ib, multi center, open label study with multiple treatment arms. The study is comprised of a dose evaluation/escalation part and a dose expansion part.

MBG453 in combination with NIS793 will be explored as the initial backbone. As the study progresses and based on emerging clinical data collected from this study, Novartis, in agreement with the study Investigators will decide whether or not:

* To proceed with any treatment arm that reaches recommended dose(s) to explore further the safety, tolerability, and anti-tumor activity in the dose expansion part.

* To add a third partner to comprise a triplet treatment arm in the dose evaluation/escalation part (such as Treatment Arm 2 with decitabine or Treatment Arm 3 with spartalizumab.

* To explore MBG453 single agent (Treatment Arm 4) and/or NIS793 single agent (Treatment Arm 5) in the dose expansion part in order to assess the single agent contributions to efficacy.

The patient population will include male or female adults (age 18 or over) with a confirmed diagnosis of primary myelofibrosis (PMF) as defined by the World Health Organization (WHO) criteria, or Post-Polycythemia Vera Myelofibrosis (PPV-MF), or Post-Essential Thrombocythemia Myelofibrosis (PET-MF) based on the revised IWG-MRT) criteria, irrespective of JAK2 mutation status and must have been treated with a JAK inhibitor for at least 28 days but no more than 6 months and experienced according to the Investigator suboptimal response defined by loss of spleen response, or worsening of symptoms or discontinuation due to adverse events (AE).

Data analysis: the primary objective of the study is to characterize the safety and tolerability of each combination and identify the recommended dose. The primary analysis will be based on a Bayesian Hierarchical Logistic Regression Model (BHLRM) and summaries of other safety, tolerabitliy endpoints. Efficacy will be assessed based on IWG-MRT. The study data will be analyzed and reported based on all patients' data up to the time when 80% of the patients have completed the follow-up for disease progression or discontinued the study for any reason, and all patients have completed treatment and the safety follow-up period.

Recruitment & Eligibility

Status
WITHDRAWN
Sex
All
Target Recruitment
Not specified
Inclusion Criteria

Not provided

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Exclusion Criteria

Not provided

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Study & Design

Study Type
INTERVENTIONAL
Study Design
CROSSOVER
Arm && Interventions
GroupInterventionDescription
NIS793 + MBG453MBG453treatment with NIS793 + MBG453
NIS793 + MBG453NIS793treatment with NIS793 + MBG453
NIS793 + MBG453 + SpartalizumabMBG453Treatment with NIS793 + MBG453 + Spartalizumab
NIS793 + MBG453 + SpartalizumabNIS793Treatment with NIS793 + MBG453 + Spartalizumab
NIS793 + MBG453 + DecitabineNIS793treatment with NIS793 + MBG453 + Decitabine
NIS793 + MBG453 + DecitabineMBG453treatment with NIS793 + MBG453 + Decitabine
NIS793NIS793treatment with NIS793
MBG453MBG453treatment with MBG453
NIS793 + MBG453 + DecitabineDecitabinetreatment with NIS793 + MBG453 + Decitabine
NIS793 + MBG453 + SpartalizumabSpartalizumabTreatment with NIS793 + MBG453 + Spartalizumab
Primary Outcome Measures
NameTimeMethod
Incidence of Dose limiting toxicities (DLT)12 months

A dose-limiting toxicity (DLT) is defined as a clinically relevant adverse event or abnormal laboratory value where the relationship to study treatment cannot be ruled out, and which is unrelated to disease, disease progression, intercurrent illness, or concomitant medications that occurs within the DLT monitoring period and meets any of the criteria included in Table 6-4 (Criteria for defining dose-limiting toxicities).

Incidence and severity of AEs and SAEs, including changes in laboratory values, vital signs, and ECGs36 months

Incidence and severity of AEs and SAEs, including changes in laboratory values, vital signs, and electrocardiograms (ECGs). A Serious adverse event (SAE) is defined as one of the following:

* Is fatal or life-threatening

* Results in persistent or significant disability/incapacity

* Constitutes a congenital anomaly/birth defect

* Is medically significant

* Requires inpatient hospitalization or prolongation of existing hospitalization.

Dose interruptions36 months

Tolerability measured by the number of subjects who have interruptions of study treatment and reason for interruptions

Dose reductions36 months

Tolerability measured by the number of subjects who have reductions of study treatment and reason for reductions

Dose intensity36 montths

Tolerability measured by the dose intensity of study drug, Relative Dose intensity for subjects with non-zero duration of exposure is computed as the ratio of dose intensity and planned dose intentity

Secondary Outcome Measures
NameTimeMethod
Clinical benefit rate based on revised IWG-MRT (International Working Group Myelofibrosis Research & Treatment) criteria: complete response (CR), partial response (PR), stable disease, progressive disease (PD), Anemia response, Spleen response, relapse36 months

Proportion of subjects achieving IWG-MRT (International Working Group for Myelofibrosis Research and Treatment) response criteria:

* Proportion of subjects with: complete response (CR) and partial response (PR), stable disease, progressive disease (PD).

* Proportion of subjects achieving Anemia response (anemia improvement of Hb ≥2.0 g/dL for transfusion independent subjects at baseline; or transfusion independence for transfusion dependent subjects at baseline).

* Proportion of subjects achieving spleen response (by palpation) from baseline or spleen volume reduction (by imaging) from baseline.

* Proportion of subjects experiencing relapse.

Proportion of subjects achieving improvement of Anemia36 months

Proportion of subjects achieving improvement of Hb level of ≥ 1.5 g/dL from baseline

Progression-free survial time (PFS)36 months

PFS is defined as the time from the date of start of treatment to the date of death by any cause or date of first documented progression as per IWG-MRT (International Working Group for Myelofibrosis Research and Treatment) criteria:

* Progressive splenomegaly as assessed by increasing spleen volume (by MRI/CT) of ≥ 25% from baseline.

* Accelerated phase defined by a circulating peripheral blood blast content of \> 10% but \<20% confirmed after 2 weeks.

* Deteriorating cytopenia (dCP) independent from treatment, defined for all patients by platelet count \< 35 x10\^9/L or neutrophil count \< 0.75 x10\^9/L that lasts for at least 4 weeks.

* Leukemic transformation defined by a peripheral blood blast content of ≥ 20% associated with an absolute blast count of ≥ 1x10\^9/L that lasts for at least 2 weeks or a bone marrow blast count of ≥ 20%.

Duration of response36 months

Time between the date of first documented response (as per the revised International Working Group for Myelofibrosis Research and Treatment IWG-MRT criteria) and the date of first documented progression or death.

Cmax (Maximum Concentration)36 months

The maximum observed plasma, blood serum or other body fluid drug concentration

Tmax36 months

Time to reach maximum plasma, blood serum or other body fluid drug concentration

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