Akari Therapeutics Announces Initiation of Pivotal Phase III Trial of Nomacopan in Pediatric Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy (HSCT-TMA) Following the Opening of its IND

On December 23, 2019 Akari Therapeutics, Plc (Nasdaq:AKTX), a biopharmaceutical company focused on innovative therapeutics to treat orphan autoimmune and inflammatory diseases where the complement and/or leukotriene systems are implicated, reported that a U.S. Food and Drug Administration (FDA) investigational new drug application (IND) is open for its multicenter Phase III study for the treatment of pediatric HSCT-TMA with nomacopan, allowing clinical sites to open in the first quarter of 2020 (Press release, Akari Therapeutics, DEC 23, 2019, View Source [SID1234552577]).

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"With the pediatric HSCT-TMA IND now open we look forward to starting the pivotal Phase III study of nomacopan in HSCT-TMA, a potential treatment for a high risk pediatric population that suffer very high death rates and for which there are currently no approved therapies. If successful, we expect HSCT-TMA to be a gateway into a range of other poorly treated orphan TMAs," commented Clive Richardson, CEO of Akari Therapeutics. "In addition, following the recent successful completion of our Phase II bullous pemphigoid study, we expect data from our Phase I/II atopic keratoconjunctivitis trial in early 2020 and interim data from our Phase III paroxysmal nocturnal hemoglobinuria trial in the first half of 2020."

HSCT-TMA is an orphan hematological condition that occurs in up to 30% of patients who have received a hematopoietic stem cell transplant (HSCT). There are no approved treatments for pediatric HSCT-TMA, and it has an estimated mortality rate of more than 80% in children with the severe form of the disease1. It is this severe form that is being targeted with nomacopan which is a bifunctional inhibitor of complement C5 and leukotriene B4 (LTB4). Following the recent end-of-Phase II meeting with the FDA, Akari has now opened an IND to initiate its pivotal pediatric HSCT-TMA study based on a single arm responder-based design. Recruitment will be focused on specialist pediatric sites in the U.S. and Europe where treatment tends to be concentrated in specialist centres.

Whilst the role of complement inhibition is understood to play an important role in pediatric HSCT-TMA, the Company believes LTB4 may also be an important target in reducing epithelial activation in both TMA and graft versus-host disease2 (GVHD) which often occur simultaneously. The Company believes daily dosing with nomacopan may also be of particular advantage in facilitating more complete complement suppression, especially in HSCT-TMA patients with high transfusion requirements.

As previously announced, this two-part pivotal Phase III study of nomacopan in pediatric patients with HSCT-TMA is based on guidance from the Company’s end-of-Phase II meeting with the FDA. Part A of the trial is a dose confirmation study. Part B of the trial is a single arm responder-based efficacy study that will follow an interim analysis of Part A and a meeting with the FDA. Akari has both FDA fast track and orphan status for this program.

1 Sonata Jodele, et al. New approaches in the diagnosis, pathophysiology, and treatment of pediatric hematopoietic stem cell transplantation associated thrombotic microangiopathy. Transfus Apher Sci . 2016 April; 54(2): 181–190

2 Takatsuka, et al. Predicting the severity of intestinal graft-versus-host disease from leukotriene B4 levels after bone marrow transplantation. Transplantation 2000, 26: 1313-1316

Progenics Pharmaceuticals Announces Phase 3 CONDOR Trial of PyLTM in Prostate Cancer Achieved Primary Endpoint

On December 23, 2019 Progenics Pharmaceuticals, Inc. (Nasdaq:PGNX), an oncology company developing innovative targeted medicines and artificial intelligence to find, fight and follow cancer, reported positive top line results from the Phase 3 CONDOR trial evaluating the diagnostic performance and clinical impact of PyLTM (18F-DCFPyL) in men with biochemical recurrence of prostate cancer (Press release, Progenics Pharmaceuticals, DEC 23, 2019, View Source [SID1234552574]). PyL is the Company’s PSMA-targeted small molecule positron emission tomography (PET) imaging agent designed to visualize prostate cancer.

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"There is a need for improved diagnostics for prostate cancer to replace conventional imaging tests that have limited performance characteristics, especially in men with biochemical recurrence of their disease," said Barry Siegel, MD, Professor of Radiology at Washington University School of Medicine in St. Louis. "The high positive predictive value demonstrated in this study reflects the clinical utility of PSMA-targeted PET imaging agents providing actionable information to physicians to guide treatment plans and improve disease management of one of the most prevalent cancers in the U.S."

The Phase 3 CONDOR trial is a prospective, multi-center, open label pivotal trial in which 208 patients with biochemical recurrence of prostate cancer and uninformative baseline imaging based on conventional modalities (i.e. Axumin, Choline PET, CT/MR and/or bone scan) were dosed and imaged with PyL at 14 sites in the United States and Canada. The CONDOR trial achieved its primary endpoint, with a correct localization rate (CLR) of 84.8% to 87.0% among the three blinded independent readers (the lower bound of the 95% confidence intervals ranging from 77.8% to 80.4%). CLR is based on positive predictive value, defined as the percentage of patients with a one-to-one correspondence between localization of at least one lesion identified on 18F-DCFPyL PET/CT and a composite truth standard comprised of histopathology, conventional imaging and/or changes in PSA levels following radiation therapy.

Safety results showed PyL was well tolerated, consistent with the Phase 2 OSPREY trial results. There was one serious adverse event of hypersensitivity reported in one patient as related to the study drug. The most frequent adverse event reported was headache, which was reported in four patients (1.9% of the trial population).

"The positive results of our Phase 3 CONDOR trial reinforce our belief in the potential of PyL to enable better physician treatment decisions and, ultimately, improve patient outcomes. The rapid trial enrollment and physician support further underscore the excitement and positive reception of PyL as a new addition in the fight against prostate cancer and, together with the commercial performance of diagnostic agents in use today, reinforce our belief in the significant market opportunity for PyL," said David Mims, Interim CEO. "Based on these positive results, we plan to submit a New Drug Application (NDA) to the U.S. Food and Drug Administration (FDA) for PyL in the second half of 2020."

Progenics PyL Phase 3 Top Line Page 2

Additional results from the CONDOR trial are expected to be presented at an upcoming medical meeting.

Investor Conference Call

Progenics will host a conference call today at 8:00 AM Eastern Time to discuss the results. The live and replayed webcast of the call will be available through the Company’s website at www.progenics.com. To participate in the live call by phone, dial (877) 250-8889 (USA) or (720) 545-0001 (international) and enter the passcode 9165086. The replay of the call will be available for 90 days.

About PyL for PET Imaging of Prostate Cancer

PyL (also known as 18F-DCFPyL) is a fluorinated PSMA-targeted positron emission tomography ("PET") imaging agent that enables visualization of both bone and soft tissue metastases to determine the presence or absence of recurrent and/or metastatic prostate cancer.

About Prostate Cancer

Prostate cancer is the second most common form of cancer affecting men in the United States: an estimated one in nine men will be diagnosed with prostate cancer in his lifetime. The American Cancer Society estimates that each year approximately 174,650 new cases of prostate cancer will be diagnosed and about 31,620 men will die of the disease. Approximately 2.9 million men in the U.S. currently count themselves among prostate cancer survivors.

Rubius Therapeutics Appoints Christina Coughlin, M.D., Ph.D., as Chief Medical Officer

On December 23, 2019 Rubius Therapeutics, Inc. (Nasdaq:RUBY), a clinical-stage biopharmaceutical company that is genetically engineering red blood cells to create an entirely new class of cellular medicines, reported the appointment of Christina Coughlin, M.D., Ph.D., as the Company’s chief medical officer, effective January 6, 2020 (Filing, 8-K, Rubius Therapeutics, DEC 23, 2019, View Source [SID1234552573]). With Dr. Coughlin’s appointment, Chris Carpenter, M.D., Ph.D., Rubius’ previous chief medical officer, is transitioning to a scientific advisory role.

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"As Rubius begins its next chapter as a clinical-stage organization, we are excited to welcome Christina to Rubius, as our chief medical officer. Christina brings extensive experience leading clinical development and translational medicine teams and has a track record of building successful drug development organizations, with a particular focus in cellular therapy and oncology. We look forward to having her clinical and scientific leadership as we advance our pipeline of Red Cell Therapeutics for the potential treatment of rare diseases, cancer and autoimmune diseases," said Pablo J. Cagnoni, M.D., chief executive officer. "I would like to thank Chris for his many contributions to Rubius and look forward to his continued guidance through his scientific advisory role."

A trained oncologist and immunologist, Dr. Coughlin joins Rubius from Tmunity Therapeutics, Inc., where she served as chief medical officer and was responsible for the development of Tmunity’s CAR-T and TCR-T cellular therapy pipeline across preclinical, regulatory and clinical development activities. Prior to Tmunity, Ltd., Dr. Coughlin served as chief medical officer at Immunocore, where she led the development of the preclinical and clinical pipeline of soluble T cell receptor product candidates recognizing multiple tumor targets, including advancing the lead program from Phase 1 to dual pivotal clinical trials. Earlier, she held positions at several pharmaceutical companies, including executive director of oncology clinical development at Novartis; international project team leader, early development at Morphotek, Inc.; and was an asset team leader in early clinical development at Pfizer’s Oncology Business Unit. Before entering the pharmaceutical industry, Dr. Coughlin was an instructor in pediatrics at the University of Pennsylvania School of Medicine; completed her postdoctoral research fellowship in the Translational Research Group of the Abramson Family Cancer Research Institute under the direction of CAR-T cell therapy pioneer Carl June, M.D.; and was an attending physician and instructor in the Department of Pediatrics and Division of Oncology at The Children’s Hospital of Philadelphia. She holds an M.D. and Ph.D. from the University of Pennsylvania School of Medicine and a B.S. in mathematics and biology from Temple University.

"I have dedicated my career to leading the development of potentially transformative, novel cellular therapies for the treatment of patients with cancer," said Christina Coughlin, M.D., Ph.D., chief medical officer of Rubius Therapeutics. "I believe the Rubius Therapeutics’ RED PLATFORM represents the next forefront in cellular therapy innovation and with it the potential to help patients across multiple therapeutic areas. I am excited to join the team during such a pivotal time for the company."

Karyopharm Submits New Drug Application to U.S. FDA for XPOVIO® (selinexor) as a Treatment for Patients with Relapsed or Refractory Diffuse Large B-Cell Lymphoma

On December 23, 2019 Karyopharm Therapeutics Inc. (Nasdaq:KPTI), an oncology-focused pharmaceutical company, reported it has submitted a New Drug Application (NDA) to the U.S. Food and Drug Administration (FDA) seeking accelerated approval for XPOVIO (selinexor), the Company’s first-in-class, oral Selective Inhibitor of Nuclear Export (SINE) compound, as a new treatment for patients with relapsed or refractory diffuse large B-Cell lymphoma (DLBCL) after at least two prior multi-agent therapies and who are ineligible for stem cell transplantation, including CAR-T (chimeric antigen receptor modified T cell) therapy (Press release, Karyopharm, DEC 23, 2019, View Source [SID1234552571]). XPOVIO has received both Orphan Drug and Fast Track designations from the FDA for this indication.

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"Earlier this year we reported compelling results from the Phase 2b SADAL study investigating XPOVIO in patients with relapsed or refractory DLBCL. The updated results submitted in the NDA demonstrate a 28.3% overall response rate, including an 11.8% complete response rate, and a median duration of response of over 9 months," said Sharon Shacham, PhD, MBA, Founder, President and Chief Scientific Officer of Karyopharm. "These data highlight XPOVIO’s potential as a new, first-in-class oral therapy for patients whose DLBCL has progressed following two prior treatments and who have extremely limited treatment options available. The submission of this second NDA is a significant achievement for Karyopharm and brings XPOVIO one step closer to serving an entirely new group of patients. We are sincerely grateful to all of the patients, caregivers and physicians who have contributed to the XPOVIO DLBCL program, and we look forward to providing future updates on the status of this NDA. If approved, XPOVIO would represent the first and only oral regimen to treat patients with relapsed or refractory diffuse large B-cell lymphoma."

Karyopharm also expects to submit a marketing authorization application (MAA) to the European Medicines Agency in 2020 requesting conditional approval for XPOVIO in this same indication.

About XPOVIO (selinexor)

XPOVIO is a first-in-class, oral Selective Inhibitor of Nuclear Export (SINE) compound. XPOVIO functions by selectively binding to and inhibiting the nuclear export protein exportin 1 (XPO1, also called CRM1). XPOVIO blocks the nuclear export of tumor suppressor, growth regulatory and anti-inflammatory proteins, leading to accumulation of these proteins in the nucleus and enhancing their anti-cancer activity in the cell. The forced nuclear retention of these proteins can counteract a multitude of the oncogenic pathways that, unchecked, allow cancer cells with severe DNA damage to continue to grow and divide in an unrestrained fashion. In addition to receiving accelerated U.S. Food and Drug Administration (FDA) approval of XPOVIO in July 2019 in combination with dexamethasone for the treatment of adult patients with relapsed refractory multiple myeloma (RRMM) who have received at least four prior therapies and whose disease is refractory to at least two proteasome inhibitors, at least two immunomodulatory agents, and an anti-CD38 monoclonal antibody, Karyopharm has also submitted a Marketing Authorization Application (MAA) to the European Medicines Agency (EMA) with a request for conditional approval of selinexor. A New Drug Application was recently submitted to the FDA seeking accelerated approval for selinexor as a new treatment for patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL). Selinexor has received Fast Track and Orphan designations from the FDA for the patient population evaluated in the SADAL study. Selinexor is also being evaluated in several other mid-and later-phase clinical trials across multiple cancer indications, including in multiple myeloma in a pivotal, randomized Phase 3 study in combination with Velcade (bortezomib) and low-dose dexamethasone (BOSTON), as a potential backbone therapy in combination with approved therapies (STOMP), in liposarcoma (SEAL) and in endometrial cancer (SIENDO), among others. Additional Phase 1, Phase 2 and Phase 3 studies are ongoing or currently planned, including multiple studies in combination with approved therapies in a variety of tumor types to further inform Karyopharm’s clinical development priorities for selinexor. Additional clinical trial information for selinexor is available at www.clinicaltrials.gov.

IMPORTANT SAFETY INFORMATION IN APPROVED MULTIPLE MYELOMA INDICATION

Thrombocytopenia

XPOVIO can cause thrombocytopenia, leading to potentially fatal hemorrhage. Thrombocytopenia was reported as an adverse reaction in 74% of patients, and severe (Grade 3-4) thrombocytopenia occurred in 61% of patients treated with XPOVIO. The median time to onset of the first event was 22 days. Bleeding occurred in 23% of patients with thrombocytopenia, clinically significant bleeding occurred in 5% of patients with thrombocytopenia and fatal hemorrhage occurred in <1% of patients.

Monitor platelet counts at baseline, during treatment, and as clinically indicated. Monitor more frequently during the first two months of treatment. Institute platelet transfusion and/or other treatments as clinically indicated. Monitor patients for signs and symptoms of bleeding and evaluate promptly. Interrupt and/or reduce dose, or permanently discontinue based on severity of adverse reaction.

Neutropenia

XPOVIO can cause neutropenia, potentially increasing the risk of infection. Neutropenia was reported as an adverse reaction in 34% of patients, and severe (Grade 3-4) neutropenia occurred in 21% of patients treated with XPOVIO. The median time to onset of the first event was 25 days. Febrile neutropenia was reported in 3% of patients.

Obtain neutrophil counts at baseline, during treatment, and as clinically indicated. Monitor more frequently during the first two months of treatment. Monitor patients for signs and symptoms of concomitant infection and evaluate promptly. Consider supportive measures including antimicrobials for signs of infection and use of growth factors (e.g., G-CSF). Interrupt and/or reduce dose, or permanently discontinue based on severity of adverse reaction.

Gastrointestinal Toxicity

Gastrointestinal toxicities occurred in patients treated with XPOVIO.

Nausea/Vomiting

Nausea was reported as an adverse reaction in 72% of patients, and Grade 3 nausea occurred in 9% of patients treated with XPOVIO. The median time to onset of the first nausea event was 3 days.

Vomiting was reported in 41% of patients, and Grade 3 vomiting occurred in 4% of patients treated with XPOVIO. The median time to onset of the first vomiting event was 5 days.

Provide prophylactic 5-HT3 antagonists and/or other anti-nausea agents, prior to and during treatment with XPOVIO. Manage nausea/vomiting by dose interruption, reduction, and/or discontinuation. Administer intravenous fluids and replace electrolytes to prevent dehydration in patients at risk. Use additional anti-nausea medications as clinically indicated.

Diarrhea

Diarrhea was reported as an adverse reaction in 44% of patients, and Grade 3 diarrhea occurred in 6% of patients treated with XPOVIO. The median time to onset of diarrhea was 15 days.

Manage diarrhea by dose modifications and/or standard anti-diarrheal agents; administer intravenous fluids to prevent dehydration in patients at risk.

Anorexia/Weight Loss

Anorexia was reported as an adverse reaction in 53% of patients, and Grade 3 anorexia occurred in 5% of patients treated with XPOVIO. The median time to onset of anorexia was 8 days.

Weight loss was reported as an adverse reaction in 47% of patients, and Grade 3 weight loss occurred in 1% of patients treated with XPOVIO. The median time to onset of weight loss was 15 days.

Monitor patient weight at baseline, during treatment, and as clinically indicated. Monitor more frequently during the first two months of treatment. Manage anorexia and weight loss with dose modifications, appetite stimulants, and nutritional support.

Hyponatremia

XPOVIO can cause hyponatremia; 39% of patients treated with XPOVIO experienced hyponatremia, 22% of patients experienced Grade 3 or 4 hyponatremia. The median time to onset of the first event was 8 days.

Monitor sodium level at baseline, during treatment, and as clinically indicated. Monitor more frequently during the first two months of treatment. Correct sodium levels for concurrent hyperglycemia (serum glucose >150 mg/dL) and high serum paraprotein levels. Treat hyponatremia per clinical guidelines (intravenous saline and/or salt tablets), including dietary review. Interrupt and/or reduce dose, or permanently discontinue based on severity of adverse reaction.

Infections

In patients receiving XPOVIO, 52% of patients experienced any grade of infection. Upper respiratory tract infection of any grade occurred in 21%, pneumonia in 13%, and sepsis in 6% of patients. Grade ≥3 infections were reported in 25% of patients, and deaths resulting from an infection occurred in 4% of patients. The most commonly reported Grade ≥3 infections were pneumonia in 9% of patients, followed by sepsis in 6%. The median time to onset was 54 days for pneumonia and 42 days for sepsis. Most infections were not associated with neutropenia and were caused by non-opportunistic organisms.

Neurological Toxicity

Neurological toxicities occurred in patients treated with XPOVIO.

Neurological adverse reactions including dizziness, syncope, depressed level of consciousness, and mental status changes (including delirium and confusional state) occurred in 30% of patients, and severe events (Grade 3-4) occurred in 9% of patients treated with XPOVIO. Median time to the first event was 15 days.

Optimize hydration status, hemoglobin level, and concomitant medications to avoid exacerbating dizziness or mental status changes.

Embryo-Fetal Toxicity

Based on data from animal studies and its mechanism of action, XPOVIO can cause fetal harm when administered to a pregnant woman. Selinexor administration to pregnant animals during organogenesis resulted in structural abnormalities and alterations to growth at exposures below those occurring clinically at the recommended dose.

Advise pregnant women of the potential risk to a fetus. Advise females of reproductive potential and males with a female partner of reproductive potential to use effective contraception during treatment with XPOVIO and for 1 week after the last dose.

ADVERSE REACTIONS IN APPROVED MULTIPLE MYELOMA INDICATION

The most common adverse reactions (incidence ≥20%) are thrombocytopenia, fatigue, nausea, anemia, decreased appetite, decreased weight, diarrhea, vomiting, hyponatremia, neutropenia, leukopenia, constipation, dyspnea, and upper respiratory tract infection.

The treatment discontinuation rate due to adverse reactions was 27%; 53% of patients had a reduction in the XPOVIO dose, and 65.3% had the dose of XPOVIO interrupted. The most frequent adverse reactions requiring permanent discontinuation in 4% or greater of patients who received XPOVIO included fatigue, nausea, and thrombocytopenia. The rate of fatal adverse reactions was 8.9%.

Please see XPOVIO Full Prescribing Information available at www.XPOVIO.com.

Further Information About Potential Accelerated Approval for XPOVIO in DLBCL

The FDA instituted its Accelerated Approval Program to allow for expedited approval of drugs that treat serious conditions and that fill an unmet medical need based on a surrogate endpoint or an intermediate clinical endpoint thought to predict clinical benefit, like overall response rate (ORR). Accelerated approval is available only for drugs that provide a meaningful therapeutic benefit over existing treatments at the time of consideration of the application for accelerated approval, which the FDA has reiterated in its feedback to the Company. Particularly in disease areas with multiple available and potential new therapies, such as DLBCL, accelerated approval carries a high regulatory threshold. Consistent with its general guidance, the FDA has noted to the Company its preference for randomized studies geared toward full approval, and has reminded the Company that accelerated approval requires patients to have exhausted all available approved therapies. FDA’s Fast Track designation is available to therapeutics treating an unmet medical need in a serious condition; the Company has received Fast Track designation from the FDA specifically for the population treated in the SADAL study. In light of this recognition that the SADAL patient population represents an unmet medical need and the positive clinical results reported in December 2018 at the American Society of Hematology (ASH) (Free ASH Whitepaper) Annual Meeting, and then updated results resented in June 2019 at the International Conference on Malignant Lymphoma, the Company believes that the SADAL study should support its request to the FDA for accelerated approval.

Eagle Pharmaceuticals to Present at the 38th Annual J.P. Morgan Healthcare Conference

On December 23, 2019 Eagle Pharmaceuticals, Inc. (Nasdaq:EGRX) ("Eagle" or the "Company") reported that Scott Tarriff, Chief Executive Officer, and Pete Meyers, Chief Financial Officer, will present at the 38th Annual J.P. Morgan Healthcare Conference as follows (Press release, Eagle Pharmaceuticals, DEC 23, 2019, View Source [SID1234552569]):

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Date:

Wednesday, January 15, 2020

Time:

4:00 p.m. Pacific Standard Time

Location:

Westin St. Francis Hotel, San Francisco

Webcast:

View Source

The presentation will be webcast live at the aforementioned time, and archived for 30 days thereafter, via the Company’s website at www.eagleus.com, under the Investors Section.