Targeted therapies for people of all ages

On June 12, 2023 Day One biopharmaceuticals presented its corporate presentation (Presentation, Day One, JUN 12, 2023, View Source [SID1234632654]).

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New Data Presented at EHA Show Chugai’s Subcutaneously Administered Crovalimab Achieved Disease Control and was Well-Tolerated in People with Paroxysmal Nocturnal Hemoglobinuria (PNH)

On June 12, 2023 Chugai Pharmaceutical Co., Ltd. (TOKYO: 4519) reported that positive results from the global phase III COMMODORE 1 and 2 studies, evaluating the efficacy and safety of crovalimab, an investigational, novel anti-C5 recycling monoclonal antibody, compared to eculizumab, one of the current standard of care in paroxysmal nocturnal hemoglobinuria (PNH) were presented at the European Hematology Association (EHA) (Free EHA Whitepaper) Hybrid Congress, taking place in Frankfurt, Germany on 8-11 June 2023 (Press release, Chugai, JUN 12, 2023, View Source [SID1234632652]).

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"Crovalimab, which is administered subcutaneously every four weeks, is expected to decrease the treatment burden of patients with PNH, possibly reducing dosing time and bringing the option of self-administration at home," said Chugai’s President and CEO, Dr. Osamu Okuda. "We will continue to work with Roche towards global filing for this drug so that the benefits of crovalimab can be delivered as soon as possible to patients and caregivers who are waiting for this new treatment."

PNH is a rare and life-threatening blood condition in which red blood cells are destroyed by the complement system – part of the innate immune system – causing symptoms such as anemia, fatigue, blood clots, and kidney disease.1 C5 inhibitors are known to be effective in treating the condition.2 Chugai’s Recycling Antibody technology has been applied to crovalimab and re-engineered to be recycled within the bloodstream, enabling sustained complement inhibition through low-dose subcutaneous administration every four weeks.3,4

In the COMMODORE 2 study, 79.3% (95% CI: 72.9, 84.5) of participants treated with crovalimab achieved hemolysis control from week five to week 25 compared with 79.0% (95% CI: 69.7, 86.0) with eculizumab. Additionally, 65.7% (95% CI: 56.9, 73.5) achieved transfusion avoidance (TA) from baseline to week 25 with crovalimab and 68.1% (95% CI: 55.7, 78.5) with eculizumab. TA is defined as people who become concentrated red blood cell transfusion-free and do not require transfusion per protocol-specified guidelines. Blood transfusion requirements are important clinical measures of hemolysis caused by complement dysregulation in PNH. A clinically meaningful improvement in FACIT-Fatigue score (improvement of over 5 points), which is a patient-reported scale to measure fatigue, from baseline to week 25 occurred in both arms, with an adjusted mean change of 7.8 (95% Cl: 6.5, 9.1) with crovalimab, and 5.2 (95% Cl: 3.4, 6.9) with eculizumab.5

Adverse events (AEs) occurred in 78% of participants treated with crovalimab and 80% treated with eculizumab in the COMMODORE 2 study. Serious infections occurred in 3% of participants treated with crovalimab and 7% with eculizumab, with no meningococcal infections. The most common AE that occurred in 16% of people treated with crovalimab and 13% of those treated with eculizumab was an infusion-related reaction. One participant in each arm experienced an AE that led to treatment discontinuation.5

The results from the COMMODORE 1 study indicate that crovalimab maintained disease control in people switching from currently approved complement inhibitors.6 The data supports the consistent benefit–risk profile of crovalimab, as well as subcutaneous administration with the option to self-administer, as seen in the COMMODORE 2 study.

Roche also presented preliminary data from the COMMODORE Burden of Illness study. The data suggest that despite currently available C5 inhibitor treatments, people with PNH continue experiencing a diminished quality of life and considerable costs, and they may benefit from new treatment options.7

Global phase III data from the COMMODORE 1 and 2 studies in PNH will be submitted to regulatory authorities around the world. Positive data from a third phase III study evaluating crovalimab in PNH, the COMMODORE 3 study in China, were presented at the American Society of Hematology (ASH) (Free ASH Whitepaper) Annual Meeting and Exposition on 10 December 2022. Data from the COMMODORE 3 study have been submitted via China’s Centre for Drug Evaluation Breakthrough Therapy Designation pathway. This submission has been accepted under Priority Review for approval consideration by China’s National Medical Products Administration.

About the COMMODORE 1 and 2 studies
The COMMODORE 2 study is a phase III, randomized, open-label study evaluating the efficacy and safety of crovalimab versus eculizumab in people with paroxysmal nocturnal hemoglobinuria (PNH) who have not been treated previously with C5 inhibitors. The 204 adults* enrolled in the study were randomized in a 2:1 ratio to be treated with either subcutaneous (SC) crovalimab every four weeks or intravenous (IV) eculizumab every two weeks. The six participants under 18 years old were included in a descriptive arm to be treated with SC crovalimab every four weeks.8

*Including two patients aged less than 18 years old enrolled before the revision of the protocol

The COMMODORE 1 study is a phase III, randomized, open-label study evaluating the safety of crovalimab in people with PNH switching from currently approved C5 inhibitors. The study included 89 people (18 years of age or older) currently treated with eculizumab, randomized in a 1:1 ratio to be treated with either SC crovalimab every four weeks or IV eculizumab every two weeks. In a non-randomized arm, the study also included pediatrics (<18 years of age) currently treated with eculizumab, people currently treated with ravulizumab, people currently treated with off-label doses of eculizumab (higher than the approved dose for PNH: more than 900mg per dose and/or more frequently than every two weeks), or people with known mutations in the C5 gene who do not respond to current therapies.9

About the COMMODORE Burden of Illness Study
The COMMODORE Burden of Illness (BOI) study will quantify the direct medical costs (e.g., treatment and hospitalization), direct nonmedical costs (e.g., travel), and indirect costs (e.g., impact on work productivity and family burden) associated with PNH for patients and care providers and determine the impact of PNH on health-related quality of life (HRQoL), in U.K., France, and Germany.10

About crovalimab
Crovalimab is an anti-C5 recycling antibody created with Chugai’s Recycling Antibody technology. Recycling antibodies are designed to achieve pH-dependent antigen binding so that a single antibody molecule can bind with the antigen multiple times, enabling a longer efficacy compared with a conventional antibody. Crovalimab is designed to target C5, a key component of the complement system, and is expected to control complement activity. It is also expected to reduce the treatment burden for patients and their caregivers through subcutaneous administration. Since crovalimab binds to complement C5 at a different site from existing antibody drugs, it can be an effective treatment option for patients with a specific C5 gene mutation (appears in approximately 3.2% of Japanese patients with PNH), which causes existing antibody drugs not to bind to C5.3,11
In addition to PNH, clinical trials are ongoing for atypical hemolytic uremic syndrome (aHUS). Overseas, Roche is conducting trials for sickle cell disease (SCD) and lupus nephritis.

About paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by intravascular hemolysis due to complement activation. It is caused by the clonal expansion of hematopoietic stem cells, driven by acquired mutations in the PIG-A gene.12 While symptoms may vary in each individual, there are typically two types. One is symptoms attributed to the characteristic hemolysis in PNH, such as hemoglobinuria and thrombosis. The other is hematopoietic failures similar to those associated with aplastic anemia. PNH may cause complications, including chronic kidney disease and pulmonary hypertension. In Japan, PNH is a rare disease that is listed as one of the designated intractable diseases (designated intractable disease 62). 959 individuals have been granted the medical care recipient certificate for PNH as of the end of 2021.

Company presentation

On June 12, 2023 Bluebird Bio presented its corporate presentation (Presentation, bluebird bio, JUN 12, 2023, View Source [SID1234632648]).

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AIM ImmunoTech Outlines Recent Significant Progress Across Clinical Development Pipeline and Provides Update on Positive Pre-Clinical and IP Developments

On June 12, 2023 AIM ImmunoTech Inc. (NYSE American: AIM) ("AIM" or the "Company"), an immuno-pharma company focused on the research and development of therapeutics to treat multiple types of cancers, immune disorders and viral diseases, reported an update on its ongoing clinical development programs evaluating Ampligen (rintatolimod), a dsRNA and highly selective TLR3 agonist immune-modulator with broad spectrum activity (Press release, AIM ImmunoTech, JUN 12, 2023, View Source [SID1234632642]).

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AIM Chief Executive Officer Thomas K. Equels commented, "We have made promising progress across multiple clinical fronts, and I am proud of the dedication of our team and their evident operational execution. Ampligen continues to demonstrate significant potential across multiple types of cancers, immune disorders and viral diseases. We are committed to advancing Ampligen’s development and are poised to achieve multiple key milestones in 2023."

Ampligen Phase 2 Study as a Therapy for Locally Advanced Pancreatic Cancer ("LAPC") (AMP-270)

The AMP-270 clinical trial is the Company’s randomized, open-label, controlled, parallel-arm study with the primary objective of comparing the efficacy of Ampligen versus a no treatment control group following FOLFIRINOX for subjects with locally advanced pancreatic adenocarcinoma. Secondary objectives include comparing safety and tolerability. AMP-270 is expected to enroll approximately 90 subjects in up to 30 centers across the United States and Europe.

The Company is recruiting patients for its AMP-270 Phase 2 study of Ampligen as a therapy for LAPC. The lead site at the University of Nebraska Medical Center is now open and actively working to enroll patients. The Gabrail Cancer & Research Center in Canton, Ohio is also recruiting patients.

Kelsey Klute, MD, Medical Director of the Pancreatic Diseases Specialty Clinic at Nebraska Medicine, commented, "We are pleased to join into what we believe is an important Phase 2 study that has the potential to address a much-needed treatment option for LAPC. Our team is actively working to enroll and treat patients on the study in a joint effort with all other clinical trial sites. We are encouraged by the potential of Ampligen and look forward to further exploring its potential in the treatment of LAPC."

AIM continues its efforts to open additional clinical sites at premier cancer centers across the United States and Europe. The Company remains optimistic for first patient enrollment in the second quarter of this year.

For more information about the AMP-270 please visit ClinicalTrials.gov and reference identifier NCT05494697.

Ampligen Phase 1 Study for the Treatment of Early-Stage Triple Negative Breast Cancer ("TNBC")

Ampligen is currently being evaluated in a Phase 1 study for TNBC by Roswell Park Comprehensive Cancer Center. The study is designed to evaluate the safety and tolerability of a combination of Ampligen and celecoxib with or without Intron A, when given along with chemotherapy. The primary endpoint was safety and tolerability. Secondary endpoints included pCR rate. Tumor and blood biomarkers were analyzed in exploratory studies. The goal of this approach is to increase survival. In November 2022, Roswell presented positive data at the SITC (Free SITC Whitepaper) 37th Annual Meeting demonstrating the chemokine-modulating regimen including Ampligen was well tolerated, with promising clinical activity of pathologic complete response (pCR) + microinvasive residual disease (ypTmic).

Roswell has now completed the study and is currently analyzing data. Based on current timelines, the Company anticipates Roswell will release – via publications – full study results before the end of the year.

For more information about the Phase 1 study of Ampligen for the treatment of TNBC, visit ClinicalTrials.gov and reference identified NCT04081389.

Ampligen Analysis Demonstrating Potential as a Maintenance Therapy After Systemic Chemotherapy in Patients with Metastatic and LAPC

A new analysis was recently presented in an abstract titled, "Immune Response in Stable Pancreatic Cancer after Rintatolimod Treatment," by Professor C.H.J. van Eijck, MD, PhD of Erasmus Medical Center at the 2023 Annual Pancreas Club Meeting held in Chicago, Illinois. Findings from the analysis demonstrated that treatment of patients with LAPC after FOLFIRINOX with Ampligen (rintatolimod) may induce markers of dendritic cells and T cells in a subgroup of patients. The absence of these markers may predict tumor progression after FOLFIRINOX, providing the future possibility of identifying pancreatic cancer patients who could potentially respond to Ampligen.

The results from the new analysis build on the previously reported peer-reviewed article "Rintatolimod (Ampligen) Enhances Numbers of Peripheral B Cells and Is Associated with Longer Survival in Patients with Locally Advanced and Metastasized Pancreatic Cancer Pre-Treated with FOLFIRINOX: A Single-Center Named Patient Program," published in March 2022 in the journal Cancers.

Ampligen as a Potential Therapy Against Ebola Virus Disease ("EVD")

The South African Patent and Trademark Office (CIPC) has granted patent No. 2022/01079, titled "Compositions and Methods Useful for Ebola Virus Infection." This pioneering patent recognizes the efficacy of AIM’s tdsRNA drug family, of which Ampligen is a member, as a potential solution to combat the devastating impact of Ebola outbreaks. Under the newly granted patent, a composition comprising tdsRNA may be used in a method that involves administering the composition to a subject to prevent, treat, inhibit, or attenuate an Ebola virus infection in that subject. Significantly, the composition may be used as a treatment for individuals already infected with the Ebola virus, or the composition may be used to prevent an Ebola infection.

About Ampligen

Ampligen is AIM’s dsRNA product candidate being developed for globally important cancers, viral diseases and disorders of the immune system. Ampligen has demonstrated in the clinic the potential for standalone efficacy in a number of solid tumors. Additionally, Ampligen has shown success in increasing survival rates and efficacy in the treatment of animal tumors when used in combination with checkpoint blockade therapies.

Ampligen is currently being evaluated as a combinational therapy for the treatment of a variety of solid tumor types in multiple clinical trials – both underway and planned – at major cancer research centers around the country. Ampligen is being used to treat pancreatic cancer patients in an Early Access Program approved by the Inspectorate of Healthcare in the Netherlands at Erasmus Medical Center. Additionally, Ampligen is also approved in Argentina for the treatment of severe chronic fatigue syndrome and is currently being evaluated in SARS-CoV-2/COVID-19, myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) and Post COVID Conditions.

Chinook Therapeutics Enters into Agreement to be Acquired by Novartis AG

On June 11, 2023 Chinook Therapeutics, Inc. (Nasdaq: KDNY), a biopharmaceutical company focused on the discovery, development and commercialization of precision medicines for kidney diseases, reported that it has entered into an agreement and plan of merger with Novartis AG pursuant to which Novartis will acquire Chinook for $40 per share in cash, or a total of $3.2 billion (Press release, Chinook Therapeutics, JUN 11, 2023, View Source [SID1234632641]). This offer represents a premium of 83 percent to Chinook’s 60-day volume-weighted average stock price and 67 percent to Chinook’s closing price on June 9, 2023. In addition, Chinook shareholders will receive contingent value rights (CVRs) providing for payment of up to $4 per share upon the achievement of certain future regulatory milestones with respect to Chinook’s lead product candidate, atrasentan. Total consideration including the contingent value right, if the milestones are achieved, would be approximately $3.5 billion. The transaction has been unanimously approved by the Boards of Directors of both companies.

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"We are pleased that Novartis recognizes the significant value that the Chinook team has built with our pipeline of clinical and preclinical programs for patients with rare, severe chronic kidney diseases," said Eric Dobmeier, president and chief executive officer of Chinook Therapeutics. "We believe this transaction is great news for kidney disease patients and the programs we have built at Chinook. Through this merger, Novartis can apply its substantial resources to pursue broader development efforts and commercialization of atrasentan, zigakibart (BION-1301) and other programs in our pipeline to build its global renal therapeutic area."

Completion of the transaction is expected in the second half of 2023, pending approval by Chinook’s stockholders and satisfaction of other customary closing conditions. Until that time, Chinook will continue to operate as a separate and independent company.

Centerview Partners LLC and MTS Health Partners, L.P. are serving as financial advisors, and Fenwick & West LLP is serving as legal counsel to Chinook.

Transaction Details

Under the terms of the merger agreement, Novartis will acquire all of the outstanding shares of Chinook through a subsidiary for a price of $40 per share in cash at closing. The CVRs to be issued to Chinook shareholders will provide for payments of up to an additional $4 per share with respect to specific regulatory approvals for atrasentan, $2 of which is related to IgA nephropathy and $2 of which is related to focal segmental glomerulosclerosis. The closing of the proposed transaction is subject to certain conditions, including approval by Chinook’s stockholders, the expiration of the waiting period under the Hart-Scott-Rodino Antitrust Improvements Act and other customary conditions. A copy of the merger agreement will be filed with the Securities and Exchange Commission ("SEC") and will be publicly available.