BridgeBio Pharma’s Affiliate QED Therapeutics and Partner Helsinn Group Announce FDA Approval of TRUSELTIQ™ (infigratinib) for Patients with Cholangiocarcinoma

On June 1, 2021 BridgeBio Pharma, Inc. (Nasdaq: BBIO), through its affiliate QED Therapeutics, Inc., and Helsinn Group reported that the US Food and Drug Administration (FDA) has approved TRUSELTIQ (infigratinib) under the accelerated approval program for the treatment of patients with previously-treated locally advanced or metastatic cholangiocarcinoma (CCA) harboring an FGFR2 fusion or rearrangement (Press release, BridgeBio, JUN 1, 2021, View Source [SID1234583333]). TRUSELTIQ is an orally administered, ATP-competitive, tyrosine kinase inhibitor of FGFR. In the pivotal trial of patients with advanced, unresectable CCA, an aggressive malignancy with poor prognosis, TRUSELTIQ led to cases of tumor shrinkage. CCA is known to affect approximately 20,000 people in the United States and European Union each year and has a median five-year survival rate of only 9%.1

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"This is an important milestone for patients diagnosed with FGFR2-fusion-driven cholangiocarcinoma who have recurred after first-line therapy and are in need of targeted options for further treatment," said Susan Moran, M.D., M.S.C.E., Chief Medical Officer for QED. "Based on the efficacy seen to date, our team believes infigratinib possesses promise for a range of FGFR-driven conditions, including other cancers. We will continue to evaluate its safety and efficacy in these areas of unmet need."

The approval of TRUSELTIQ is based on a Phase 2 clinical study in which 108 patients who had undergone at least one prior treatment for advanced CCA received 125 mg of TRUSELTIQ daily for 21 days of 28-day cycles. Of these patients, 107 (99%) had Stage IV CCA. All patients had received at least 1 prior line of systemic therapy. The study’s primary endpoint demonstrated a confirmed objective response rate (ORR) of 23% (95% CI 16-32%). The study also showed a median duration of response (DOR) of 5.0 months (95% CI 3.7–9.3 months). Common adverse reactions and laboratory abnormalities (of >30%) were increased creatinine, increased phosphate, decreased phosphate, nail toxicity, stomatitis, increased alkaline phosphatase, decreased hemoglobin, increased alanine aminotransferase, dry eye, fatigue, increased lipase, decreased lymphocytes, increased calcium, decreased sodium, alopecia, increased triglycerides, increased aspartate aminotransferase, decreased platelets, increased urate, palmar-plantar erythrodysesthesia syndrome, arthralgia, and dysgeusia. Please see below for additional important safety information for TRUSELTIQ.

The above data were presented at the 2021 American Society of Clinical Oncology (ASCO) (Free ASCO Whitepaper) Gastrointestinal Cancers Symposium by the lead investigator, Milind Javle, M.D., Professor of Gastrointestinal Medical Oncology at The University of Texas MD Anderson Cancer Center.

"While targeted treatments have extended survival for many types of cancer, people diagnosed with cholangiocarcinoma have previously been presented with extremely limited treatment options coupled with low statistical survival data," said Dr. Javle. "In this study, TRUSELTIQ showed promise as a targeted treatment option for patients with FGFR2-fusion-driven cholangiocarcinoma with a well-tolerated safety profile in line with previous observations in this patient population."

"The approval of TRUSELTIQ provides a new and exciting treatment option for patients with CCA harboring an FGFR2 fusion," said Stacie Lindsey, Chief Executive Officer of the Cholangiocarcinoma Foundation. "We appreciate the fact that there is a robust patient support program, ForgingBridges, to help patients access care and support them throughout their treatment journey."

Additional marketing applications for infigratinib are currently under review in Australia and Canada under Project Orbis, an initiative of the FDA’s Oncology Center of Excellence that allows for concurrent submission and review of oncology drugs among participating international regulatory agencies.

BridgeBio and Helsinn Group’s affiliate, Helsinn Therapeutics (U.S.), Inc., will be jointly responsible for commercialization activities in the U.S. and will share U.S. profits and losses on an equal basis. Helsinn Group will have exclusive commercialization rights on infigratinib outside of the U.S., excluding China, Hong Kong and Macau. BridgeBio will be eligible for tiered royalties as a percentage of adjusted net sales, and payments totaling up to approximately $2.45 billion USD in the aggregate. Helsinn Group will fund the majority of ongoing and future research and development related to infigratinib in oncology. BridgeBio and Helsinn Group entered into a global collaboration and licensing agreement in March 2021. BridgeBio previously entered a strategic collaboration with LianBio for development and commercialization of infigratinib in oncology indications in China, Hong Kong and Macau.

Paul Rittman, Chief Executive Officer of Helsinn Therapeutics, said, "Today’s FDA approval of TRUSELTIQ for patients with previously-treated locally advanced or metastatic CCA harboring an FGFR2 fusion or rearrangement provides a new therapy option for patients with a very low rate of survival. This new therapy has the potential to make a life changing impact on patients with few treatment options, and Helsinn Therapeutics looks forward to working with BridgeBio to make it widely accessible to health care providers and patients in the US."

ForgingBridges | TRUSELTIQ is a comprehensive patient support program designed specifically to provide education, access and affordability resources for patients during their TRUSELTIQ journey. For more information, visit: TRUSELTIQ.com/forgingbridges-overview.

Visit TRUSELTIQ.com for more information, including full Prescribing Information.

About TRUSELTIQ (infigratinib)

TRUSELTIQ (infigratinib) is an orally administered, ATP-competitive, tyrosine kinase inhibitor of FGFR, approved for the treatment of individuals with FGFR2 fusion-driven cholangiocarcinoma (bile duct cancer). TRUSELTIQ targets the fibroblast growth factor receptor (FGFR) protein, blocking downstream activity. In clinical studies, TRUSELTIQ demonstrated a clinically meaningful rate of tumor shrinkage (overall response rate) and duration of response. Visit TRUSELTIQ.com for more information. Infigratinib is not FDA approved for any other indication in the U.S. and is not approved for use by any other health authority. It is currently being evaluated in clinical studies for first-line cholangiocarcinoma and urothelial carcinoma (bladder cancer). For more information, visit QEDTx.com.

About Cholangiocarcinoma (CCA)

Cholangiocarcinoma, a cancer of the bile ducts of the liver, is a serious and often fatal disease which affects approximately 20,000 people in the United States and European Union each year. FGFR2 genetic aberrations are present in approximately 15% to 20% of people who have this disease. Currently, the five-year survival rate is only 9%.1 Advanced, unresectable CCA is a rare, aggressive malignancy with a poor prognosis.

Clinical Studies1

The efficacy of TRUSELTIQ was based on a single-arm Phase 2 study which included 108 patients with previously treated, unresectable locally advanced or metastatic cholangiocarcinoma with an FGFR2 fusion or rearrangement. Ninety-nine percent of patients had metastatic (Stage IV) disease at the time of study entry.

These 108 adult patients with advanced/metastatic CCA received infigratinib 125 mg orally for 21 days of each 28-day cycle until unacceptable toxicity or disease progression. All patients received prophylaxis with the oral phosphate binder sevelamer. TRUSELTIQ achieved a 23% objective response rate (ORR) and a median duration of response (DOR) of 5.0 months.

U.S. Indication for TRUSELTIQ

TRUSELTIQ is indicated for the treatment of adults with previously treated, unresectable locally advanced or metastatic cholangiocarcinoma with a fibroblast growth factor receptor 2 (FGFR2) fusion or other rearrangement as detected by an FDA-approved test.

Accelerated approval was granted based on overall response rate and duration of response. Continued approval for this indication may be contingent upon verification of clinical benefit in confirmatory trial(s).

U.S. Important Safety Information for TRUSELTIQ

Warnings and precautions

Ocular toxicity: Retinal pigment epithelial detachment (RPED), which may cause blurred vision, occurred in 11% of 351 patients treated with TRUSELTIQ, including patients with asymptomatic RPED, with a median onset of 26 days. Perform comprehensive ophthalmological exam including optical coherence tomography prior to initiating, at 1 month, at 3 months, and then every 3 months during treatment with TRUSELTIQ. Urgently evaluate patients for onset of visual symptoms and follow up every 3 weeks until resolved or TRUSELTIQ is discontinued. Withhold TRUSELTIQ as recommended. Dry eye occurred in 29% of 351 patients; treat with ocular demulcents as needed
Hyperphosphatemia and soft tissue mineralization: Hyperphosphatemia, which can lead to soft tissue mineralization, cutaneous calcinosis, non-uremic calciphylaxis, vascular calcification, and myocardial calcification, occurred in 82% of 351 patients treated with TRUSELTIQ, with a median time to onset of 8 days (range 1-349); 83% of 351 patients treated with TRUSELTIQ received phosphate binders. Monitor for hyperphosphatemia throughout treatment. Initiate phosphate-lowering therapy for serum phosphate >5.5 mg/dL; withhold TRUSELTIQ and initiate phosphate-lowering therapy for serum phosphate >7.5 mg/dL; withhold, reduce the dose, or permanently discontinue TRUSELTIQ based on duration and severity of hyperphosphatemia
Embryo-fetal toxicity: TRUSELTIQ can cause fetal harm. Advise pregnant women of the potential risk to the fetus; advise females of reproductive potential and men who are partnered with women of reproductive potential to use effective contraception during treatment with TRUSELTIQ and for 1 month after the final dose
Adverse reactions

Most common adverse reactions (incidence ≥20%, all grades): nail toxicity, stomatitis, dry eye, fatigue, alopecia, palmar-plantar erythrodysesthesia syndrome, arthralgia, dysgeusia, constipation, abdominal pain, dry mouth, eyelash changes, diarrhea, dry skin, decreased appetite, blurred vision, and vomiting
Most common laboratory abnormalities (incidence ≥20%, all grades): increased creatinine, increased phosphate, decreased phosphate, increased alkaline phosphatase, decreased hemoglobin, increased alanine aminotransferase, increased lipase, increased calcium, decreased lymphocytes, decreased sodium, increased triglycerides, increased aspartate aminotransferase (AST), increased urate, decreased platelets, decreased leukocytes, decreased albumin, increased bilirubin, and decreased potassium
Drug interactions

CYP3A inhibitors: Avoid use with strong and moderate CYP3A inhibitors
CYP3A inducers: Avoid use with strong and moderate CYP3A inducers
Gastric acid–reducing agents: Avoid coadministration with proton pump inhibitors, histamine-2 receptor antagonists (H2RA), and locally acting antacids. If coadministration of H2RA or locally acting antacids cannot be avoided, separate TRUSELTIQ administration
H2RA: Take TRUSELTIQ 2 hours before or 10 hours after
Locally-acting antacid: Take TRUSELTIQ 2 hours before or 2 hours after
Dosage and administration

Prior to initiating TRUSELTIQ: Confirm FGFR2 fusion or rearrangement; perform comprehensive ophthalmic exam including OCT; confirm negative pregnancy test in females of reproductive potential
Starting dose: Take TRUSELTIQ orally once daily on Days 1-21 of 28-day cycles; continue treatment until disease progression or unacceptable toxicity. Take TRUSELTIQ on an empty stomach with a glass of water at least 1 hour before or 2 hours after food
No renal or hepatic impairment
125 mg (one 100 mg capsule and one 25 mg capsule)
Mild and moderate renal impairment (creatinine clearance 30-89 mL/min)
100 mg (one 100 mg capsule)
Mild hepatic impairment (total bilirubin >upper limit of normal [ULN] to 1.5 x ULN or AST > ULN)
100 mg (one 100 mg capsule)
Moderate hepatic impairment (total bilirubin >1.5 to 3 x ULN with any AST)
75 mg (three 25 mg capsules)
Dose modification: Consult the TRUSELTIQ full Prescribing Information for dose modifications and monitoring recommendations for RPED, hyperphosphatemia, and other Grades 3-4 adverse reactions

Antengene Announces Fifteen Clinical Studies and Results of Selinexor to be Presented at ASCO 2021

On June 1, 2021 Antengene Corporation Limited ("Antengene", SEHK: 6996.HK), a leading innovative biopharmaceutical company dedicated to discovering, developing and commercializing global first-in-class and/or best-in-class therapeutics in hematology and oncology, reported that fifteen studies and results of selinexor, the world’s first approved oral selective inhibitor of nuclear export (SINE), will be presented at the 2021 American Society of Clinical Oncology (ASCO) (Free ASCO Whitepaper) Annual Meeting, taking place in a virtual format on June 4 to 8 (Press release, Antengene, JUN 1, 2021, View Source [SID1234583349]).

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Selected Abstracts:

Effects of weekly selinexor, bortezomib, dexamethasone (XVd) versus standard twice weekly bortezomib and dexamethasone (Vd) on RAS-mutated previously treated multiple myeloma (MM).

Abstract #: 8027

Effects of refractory status to lenalidomide on safety and efficacy of selinexor, bortezomib, and dexamethasone (XVd) versus bortezomib and dexamethasone (Vd) in patients with previously treated multiple myeloma.

Abstract #: 8024

Survival among older patients with previously treated multiple myeloma treated with selinexor, bortezomib, and dexamethasone (XVd) in the BOSTON study.

Abstract #: 8019

Updated overall survival of eltanexor for the treatment of patients with hypomethylating agent refractory myelodysplastic syndrome.

Abstract #: e19037

Results of the phase 2 MARCH Study: Oral ATG-010 (Selinexor) plus low dosedexamethasone in Chinese patients with relapsed/refractory multiple myeloma (RRMM) previously treated with an immunomodulatory agent (IMiD) and a proteasome inhibitor (PI).

Abstract #: e20002

Oral selinexor, pomalidomide, and dexamethasone (XPd) at recommended phase 2 dose in relapsed refractory multiple myeloma (MM).

Abstract #: 8018

SIENDO/ENGOT-EN5/GOG-3055: A randomized phase 3 trial of maintenance selinexor versus placebo after combination platinum-based chemotherapy in advanced or recurrent endometrial cancer.

Abstract #: TPS5610

Open-label phase 1 study evaluating the tolerability and anti-tumor activity of selinexor and pembrolizumab in colorectal cancer.

Abstract #: e15579

A phase 1/2 study of selinexor in combination with standard of care therapy for newly diagnosed or recurrent glioblastoma.

Abstract #: TPS2071

A phase Ib/II study of selinexor in combination with imatinib in patients with advanced gastrointestinal stromal tumor (GIST): SeliGIST/GEIS-41 trial.

Abstract #: 11534

Selinexor in combination with weekly paclitaxel in patients with advanced or metastatic solid tumors: Results of an open label, single-center, multiarm phase 1b study.

Abstract #: 5565

Once weekly selinexor, carfilzomib, and dexamethasone (XKd) in carfilzomib nonrefractory multiple myeloma (MM) patients.

Abstract #: 8038

A randomized, open-label, phase 3 study of low-dose selinexor and lenalidomide (Len) versus len maintenance post autologous stem cell transplant (ASCT) for newly diagnosed multiple myeloma (NDMM): ALLG MM23, Sealand.

Abstract #: TPS8055

Molecular predictors of response to selinexor in advanced unresectable de-differentiated liposarcoma (DDLS).

Abstract #: 11509

Selinexor containing regimens in patients with multiple myeloma (MM) previously treated with anti-CD38 monoclonal antibodies (αCD38 mAbs).

Abstract #: e20020

Immutep enters into a New Collaboration with Merck KGaA, Darmstadt, Germany for LAG-3 Therapy, Efti

On June 1, 2021 Immutep Limited (ASX: IMM; NASDAQ: IMMP) ("Immutep" or "the Company"), a biotechnology company developing novel immunotherapy treatments for cancer and autoimmune disease, reported a new collaboration and supply agreement with Merck KGaA , Darmstadt, Germany for a Phase I/IIa clinical trial in patients with solid tumours, called INSIGHT-005 (Press release, Immutep, JUN 1, 2021, View Source [SID1234583365]).

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The trial will evaluate the feasibility, safety and efficacy of Immutep’s lead product candidate, eftilagimod alpha (efti or IMP321), when given in combination with bintrafusp alfa (M7824), an investigational bifunctional fusion protein immunotherapy being jointly developed by Merck, Darmstadt, Germany and GlaxoSmithKline. Bintrafusp alfa aims to block two immunosuppressive pathways, TGF-ß and PD-L1, while efti activates antigen presenting cells, via the LAG-3 – MHC II pathway.

"Through INSIGHT-005, we plan to explore the effect of releasing the brakes and pushing the accelerator of the body’s immune system in three different positions of the cancer immunity cycle. The new trial builds on our knowledge and the encouraging data from the INSIGHT trial of efti, also in solid tumours. We are excited about this new clinical collaboration which allows us to extend and strengthen our relationship with an existing partner in a new and exciting setting, particularly at a time when there is growing awareness and validation of the LAG-3 MHC class II interaction," said Immutep CEO Marc Voigt.

INSIGHT-005 will be conducted by the Institute of Clinical Cancer Research, at Krankenhaus Nordwest in Frankfurt, Germany (IKF) as an investigator-initiated trial, with Prof. Dr. Salah-Eddin Al-Batran as lead investigator. The study will be run as an amendment to the protocol of the ongoing Phase I INSIGHT trial as the fifth arm (Stratum E). Prof. Al-Batran is also the lead investigator of INSIGHT and INSIGHT-004 and a member of Immutep’s clinical advisory board.

"We are very pleased to be expanding our involvement with Immutep to explore efti in a new combination with bintrafusp alfa. Our experience and knowledge of efti, combined with our extensive nationwide network of more than 500 German clinical facilities, means we are well equipped to lead the INSIGHT-005 study," said Prof. Al-Batran, lead investigator of INSIGHT-005.

INSIGHT-005 is planned to take place in 2 clinical sites in Germany. Immutep is financially supporting the trial to be run by IKF, and Merck, Darmstadt, Germany is financially contributing to the biomarker-related work. Subject to regulatory and ethics committee approval, the first patient is expected to be enrolled in mid-2021 and the trial is expected to deliver first data in early 2022.

Instil Bio to Present at the Jefferies Virtual Healthcare Conference

On June 1, 2021 Instil Bio, Inc. ("Instil") (Nasdaq: TIL), a clinical-stage biopharmaceutical company focused on developing tumor infiltrating lymphocyte, or TIL, therapies for the treatment of patients with cancer, reported that management will present a company overview at the Jefferies Virtual Healthcare Conference on Friday, June 4, 2021 at 9:30 a.m. ET (Press release, Instil Bio, JUN 1, 2021, View Source [SID1234583998]).

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A live webcast of the presentation may be accessed at: View Source or by visiting the News & Events section of the Instil Bio website at www.instilbio.com. An archived replay of the webcast will be available on the Company’s website for approximately 90 days following the presentation.

Macrophage Pharma Appoints Dr Venkat Reddy as Chief Executive Officer

On June 1, 2021 Macrophage Pharma Limited (‘MPL’), a company focused on the discovery and early stage development of highly novel small molecule therapeutics with the potential to transform outcome in inflammation, autoimmune disease and cancer through regulation of innate immune response, reported that its Chief Scientific Officer (CSO), Dr Venkat Reddy PhD, has been appointed by the Board as Chief Executive Officer (CEO) (Press release, Macrophage Pharma, JUN 1, 2021, View Source [SID1234583301]).

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Commenting on the appointment, Dr Michael Moore, Chairman of Macrophage Pharma, said: "The Board is delighted to have promoted Venkat to CEO. Since joining the Company, Venkat’s influence in combining a strategic biology focus on targets critical for regulated cell death in immune-mediated diseases with the Company’s drug discovery platform based on proprietary Esterase Sensitive Motif (ESM) technology, has been transformative, enhancing the opportunity for commercialisation of our novel small molecule assets for a variety of protein targets and therapeutically unmet diseases."
Dr Venkat Reddy, CEO of Macrophage Pharma, commented: "I am honoured to have been appointed CEO of Macrophage Pharma. The potential of our ESM technology platform to generate novel drugs is compelling as demonstrated by its ability to impact adaptive immunity through selective modulation of myeloid derived innate immune cells. I look forward to continuing my work with Macrophage Pharma’s executive team and Board of Directors to unlock the full therapeutic and commercial potential of the technology across a spectrum of human diseases."

Venkat Reddy has a successful track record and extensive strategic and management experience from senior executive roles in both European and US biotech and pharma, including Glenmark Pharmaceuticals (SVP, Global Head of Translational Sciences), Pfizer (Senior Director, Strategic Alliances and Partnerships, Centers for Therapeutic Innovation), Sanofi (Senior Director, Head, Bio-Innovation France: Global Biotherapeutics) and Novartis (Group leader, GNF). Venkat holds a PhD from the Ludwig-Maximilians Universität München and undertook post-doctoral studies in immunology and oncology at the Scripps Research Institute. He has served Macrophage Pharma as Chief Scientific Officer since January 2020.