Aura Biosciences Presents Updated AU-011 Clinical Data at ARVO 2020

On June 12, 2020 Aura Biosciences, a clinical-stage biopharmaceutical company developing a novel class of tumor targeted therapies for initial application in primary tumors such as choroidal melanoma, reported the virtual presentation of updated clinical data from its ongoing Phase 1b/2 clinical trial evaluating the safety and efficacy of light-activated AU-011, the Company’s lead product candidate for the first line treatment of primary choroidal melanoma, as part of the Association for Research in Vision and Ophthalmology (ARVO) 2020 Annual Meeting (Press release, Aura Biosciences, JUN 12, 2020, View Source [SID1234561036]).

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"The clinical data presented this year at ARVO show that AU-011 has a favorable preliminary safety profile, controls tumor growth rate, and preserves vision in the vast majority of patients, including those at high risk for vision loss with tumors close to the fovea or optic nerve," said Prithvi Mruthyunjaya, MD, MHS, Associate Professor of Ophthalmology and Director, Ocular Oncology Service, Byers Eye Institute at Stanford University, and presenter of the abstract. "We believe these data support further clinical investigation in pivotal studies."

Updated Results from the Phase 1b/2 Study Evaluating AU-011

This open-label, multicenter trial is designed to investigate single and multiple ascending doses of light-activated AU-011, administered via intravitreal injection, in adult subjects with clinically diagnosed primary choroidal melanoma. The updated data demonstrate that multiple administrations of light-activated AU-011 were generally well-tolerated. Among the patients evaluated for safety (n=56), the most common treatment-related adverse events (AEs) were expected and included vitreous inflammation, anterior chamber inflammation and increase in intraocular pressure; all were manageable with standard-of-care treatments and the majority resolved without clinical sequelae. There were two treatment-related serious AEs (vision loss; 3.6%). Tumor control and vision preservation data continue to be supportive of the planned pivotal study.

Expanding Development of AU-011 to Include Suprachoroidal Administration

In addition to intravitreal administration, Aura is also planning to investigate AU-011 using the suprachoroidal (SC) route of administration. Aura believes that delivering AU-011 into the suprachoroidal space (SCS) within the eye, has the potential to maximize bioavailability at the tumor site and could allow for the treatment of a wider range of tumor sizes (small to medium size tumors), and therefore, a larger number of patients. The Company is partnered with Clearside Biomedical for use of Clearside’s SCS Microinjector for administration of AU-011 into the SCS. Preclinical research regarding the ocular distribution and efficacy in a rabbit model of AU-011 using the SC administration is also being presented as part of the ARVO 2020 virtual program. The data showed excellent distribution of AU-011 in the SCS and complete necrosis of tumors following laser activation in a rabbit model of choroidal melanoma. Further preclinical studies are currently ongoing and Aura expects to initiate a Phase 2 clinical study evaluating SC delivery of AU-011 during the third quarter of 2020.

Cadmus Rich, MD, MBA Chief Medical Officer and Head of Research and Development at Aura Biosciences commented, "The suprachoroidal delivery route is exciting because it offers certain potential advantages over intravitreal injection, including high bioavailability of drug to the posterior segment of the eye where the choroidal melanoma is located and minimal exposure of non-targeted tissues which, based on preclinical toxicology studies, may lead to less intraocular inflammation and an improved safety profile. We believe that the SC administration of AU-011 will ultimately allow us to treat a larger number of patients with choroidal melanoma. We look forward to initiating a first-in-human Phase 2 study to assess this additional route of administration this year."

Details for the ARVO 2020 presentation is as follows:

Title: An Ongoing Phase 1b/2 Open-Label Clinical Trial to Evaluate the Safety and Efficacy of AU-011 for the Treatment of Choroidal Melanoma – Study Update

Presenter: Prithvi Mruthyunjaya, Stanford University

Abstract #: 4025

Session: Uveal melanoma

URL: View Source

Title: Ocular distribution and efficacy after suprachoroidal injection of AU-011 for treatment of ocular melanoma

Presenter: Anneli Savinainen, Aura Biosciences

Abstract #: 3615

Session: Uveal melanoma – Advances in Therapy

URL: View Source

The presentations can be accessed by ARVO members on the ARVOLearn website. The presentations can also be accessed by visiting the "Presentations" section of "News and Publications" page of the Aura Biosciences website.

About Choroidal Melanoma

Choroidal melanoma is a rare and aggressive type of eye cancer. Choroidal melanoma is the most common primary intraocular tumor in adults and develops in the uveal tract of the eye. No targeted therapies are available at present, and current radiotherapy treatments can be associated with severe visual loss and other long-term sequelae such as dry eye, glaucoma, cataracts and radiation retinopathy. The most common current treatment is plaque radiotherapy, which involves surgical placement of a radiation device on the exterior of the eye over the tumor. The alternative is enucleation, or total surgical removal of the eye. Choroidal melanoma metastasizes in approximately 50 percent of cases with liver involvement in 80-90% of cases and, unfortunately, metastatic disease is universally fatal (source: OMF). There is a very high unmet need for a new vision sparing targeted therapy that could enable early treatment intervention for this life-threatening rare disease given the lack of approved therapies, and the comorbidities of radioactive treatment options.

About Light-Activated AU-011

AU-011 is a first-in-class targeted therapy in development for the treatment of primary choroidal melanoma. The therapy consists of proprietary viral-like particle bioconjugates (VPB) that are activated with an ophthalmic laser. The VPBs bind selectively to unique receptors on cancer cells in the eye and are derived from technology originally pioneered by Dr. John Schiller of the Center for Cancer Research at the National Cancer Institute (NCI), recipient of the 2017 Lasker-DeBakey Award. Upon activation with an ophthalmic laser, the drug rapidly and specifically disrupts the cell membrane of tumor cells while sparing key eye structures, which may allow for the potential of preserving patients’ vision and reducing other long-term complications of radiation treatment. AU-011 can be delivered using equipment commonly found in an ophthalmologist’s office and does not require a surgical procedure, pointing to a potentially less invasive, more convenient therapy for patients and physicians. AU-011 for the treatment of choroidal melanoma has been granted orphan drug and fast track designations by the U.S. Food and Drug Administration and is currently in clinical development.

Onconova Therapeutics Announces Presentation at the 25th Congress of the European Hematology Association (EHA)

On June 12, 2020 Onconova Therapeutics, Inc. (NASDAQ: ONTX), a Phase 3 stage biopharmaceutical company focused on discovering and developing novel products to treat cancer, with an initial focus on myelodysplastic syndromes (MDS), reported updated aggregated baseline genomic data from HMA-failure patients screened for the INSPIRE trial were presented in an e-poster at the virtual 25th Annual EHA (Free EHA Whitepaper) Congress (Press release, Onconova, JUN 12, 2020, View Source [SID1234561035]).

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"There are more than 45 driver mutations that have been identified in higher-risk (HR) MDS patients. To our knowledge, this mutational analysis from the INSPIRE trial is among the largest such datasets to be collected," said Steven M. Fruchtman, M.D., President and Chief Executive Officer.

The on-demand presentation will be available on the EHA (Free EHA Whitepaper) website as of Friday, June 12 at 8:30 a.m. CEST, and will be accessible until October 15, 2020.

Abstract #EP829. "Mutations in RAS Pathway Genes Correlate with Type of Failure to Azacitidine: Genomic Analysis at Randomization onto the Inspire Trial."

As an exploratory endpoint, bone marrow samples were collected at baseline, months 2, 4 and 6, and every 6 months thereafter, as well as at the end of treatment, for mutational analysis. Genomic DNA was extracted and deep sequencing of 295 genes will be performed on these samples following analysis of the primary endpoint. In total, 55 different mutations were identified at baseline, with the median number of mutations per patient at 3.

"RAS pathway mutations were observed more frequently in patients that progressed on HMA therapy than those that failed HMA therapy completely. Understanding the association between RAS mutations and prognoses in this MDS setting appears to be informative to the potential role of RAS-targeting agents such as rigosertib," said Richard C. Woodman, M.D., Chief Medical Officer.

Aptose Presents Early Phase 1a/b CG-806 Clinical Findings at the 25th Congress of the European Hematology Association

On June 12, 2020 Aptose Biosciences Inc. ("Aptose" or the "Company") (NASDAQ: APTO, TSX: APS), a clinical-stage company developing highly differentiated therapeutics targeting the underlying mechanisms of cancer, reported that new clinical data on CG-806, its oral, first-in-class FLT3/BTK cluster selective kinase inhibitor, was presented in a poster presentation at the 25th Congress of the European Hematology Association (EHA) (Free EHA Whitepaper), EHA (Free EHA Whitepaper)25 Virtual Congress (Press release, Aptose Biosciences, JUN 12, 2020, View Source [SID1234561034]).

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Early Clinical Findings from a Phase 1 a/b Dose Escalation Trial to Evaluate the Safety and Tolerability of CG-806 in Patients with Relapsed or Refractory CLL/SLL or Non-Hodgkin’s Lymphomas (EHA2020 Abstract# EP711) reviewed CG-806 data for eight patients (as of the data cut-off date on May 5, 2020) with relapsed or refractory chronic lymphocytic leukemia (CLL) / small lymphocytic lymphoma (SLL) or non-Hodgkin’s lymphoma in the first in-human Phase 1 a/b, open-label, single arm, multicenter dose-escalation clinical study. The poster is available on the posters and presentations section of the Aptose website here. For more information on the ongoing study, please visit www.clinicaltrials.gov here.

CG-806 was well-tolerated in patients treated at 150mg, 300mg and 450mg BID over multiple cycles, with no drug-related dose-limiting toxicities or serious adverse events. CG-806 treatment led to lymphocytosis in two CLL patients and delivered complete inhibition of phospho-BTK and multiple oncogenic survival pathways in all patients receiving ≥ 300mg BID. Plasma from CG-806 treated patients completely inhibited phospho-FLT3 in a plasma inhibitory activity (PIA) assay, and patients receiving ≥ 300mg BID achieved steady state PK levels known to be effective in murine tumor models.

"We are pleased by evidence of CG-806’s safety and tolerability, along with early indicators of pharmacologic activity," said William G. Rice, Ph.D., Chairman, President and Chief Executive Officer. "These data continue to support further dose escalation of CG-806 and in fact, since the data cut-off date, we have progressed to the 600mg dosing cohort. The findings also suggest that dose levels evaluated in this study may be therapeutic in patients with AML."

Separately, Aptose announced it has submitted an IND for a parallel Phase 1 a/b clinical study of CG-806 in patients with relapsed or refractory FLT3-mutant or FLT3-wildtype acute myeloid leukemia (AML).

About CG-806

CG-806 is an oral, first-in-class FLT3/BTK cluster selective kinase inhibitor and is in Phase 1 clinical studies for the treatment of hematologic malignancies. This small molecule demonstrates potent inhibition of wild type and all mutant forms of FLT3 (including internal tandem duplication, or ITD, and mutations of the receptor tyrosine kinase domain and gatekeeper region), cures animals of AML in the absence of toxicity in murine xenograft models, and represents a potential best-in-class therapeutic for patients with AML and other myeloid malignancies. Likewise, CG-806 demonstrates potent, non-covalent inhibition of the wild type and Cys481Ser (C481S) mutant forms of the BTK enzyme, as well as other oncogenic kinase pathways operative in B cell malignancies, suggesting CG-806 may be developed for various B cell malignancy patients (including CLL/SLL, FL, MCL, DLBCL and others) that are resistant/refractory/intolerant to covalent or other non-covalent BTK inhibitors. Because CG-806 targets key kinases/pathways operative in malignancies derived from the bone marrow, it is in development for B-cell cancers and AML.

Karyopharm Presents XPOVIO® (selinexor) and Eltanexor Data at the European Hematology Association 2020 Virtual Annual Meeting

On June 12, 2020 Karyopharm Therapeutics Inc. (Nasdaq:KPTI), an innovation-driven pharmaceutical company, reported that eight posters relating to XPOVIO (selinexor), the Company’s first-in-class, oral Selective Inhibitor of Nuclear Export (SINE) compound, and eltanexor, its next generation SINE compound, will be presented at the European Hematology Association (EHA) (Free EHA Whitepaper) 2020 Virtual Annual Meeting taking place June 11-21, 2020 (Press release, Karyopharm, JUN 12, 2020, View Source [SID1234561033]).

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The six selinexor abstracts include: (i) overall survival data from the Phase 2b SADAL study evaluating selinexor in patients with relapsed or refractory diffuse large-B-cell lymphoma (DLBCL) demonstrating a 9-month median overall survival in a patient population in which survival is expected to be <6 months based on several historical controls, with the median overall survival not yet reached in the 29% of patients who had partial or complete responses on single agent selinexor; (ii) a post-hoc analysis from the SADAL study demonstrating clinically meaningful response rates in the subgroup of patients with primary refractory DLBCL and treated with at least two prior regimens; (iii) a post-hoc analysis from the SADAL study demonstrating durable response rates regardless of the number of prior lines of therapy or prior treatment with high dose chemotherapy with autologous stem cell transplant; (iv) an assessment of molecular markers that may predict response to selinexor in patients with DLBCL; (v) data demonstrating the anti-myeloma effects of selinexor in combination with eukaryotic translation initiation factor 4E (eIF4E); and (vi) data demonstrating selinexor’s potential to treat patients with acute myeloid leukemia harboring IDH2 pR172K mutations.

The two eltanexor abstracts include: (i) data demonstrating the efficacy of eltanexor in preclinical models of NPM1-mutated acute myeloid leukemia; and (ii) an assessment of molecular markers that may predict a response to eltanexor in patients with relapsed or refractory multiple myeloma.

"The Phase 2b SADAL study in patients with heavily pretreated DLBCL continues to generate encouraging efficacy and safety data from multiple datasets and post-hoc analyses, including a superior survival benefit over what has historically been observed with other therapies," said Sharon Shacham, PhD, MBA, President and Chief Scientific Officer of Karyopharm. "As we await the decision from the U.S. Food and Drug Administration on our supplemental new drug application expected later this month, our sales force is actively preparing for the potential launch of XPOVIO in the additional indication of relapsed or refractory DLBCL. The SADAL data presented at EHA (Free EHA Whitepaper) this year will be an important component of our physician education effort surrounding XPOVIO to treat patients with DLBCL, if approved."

Details for the EHA (Free EHA Whitepaper) 2020 virtual poster presentations are as follows:

Selinexor

Title: Survival Among Patients with Relapsed/Refractory (R/R) Diffuse Large B-Cell Lymphoma (DLBCL) Treated with Single Agent Selinexor in the SADAL Study
Lead author: Marie Maerevoet, Institut Jules Bordet
Abstract #: EP1260
Session: 19. Aggressive Non-Hodgkin Lymphoma – Clinical

Title: Efficacy and Safety of Single Agent Oral Selinexor in Patients with Primary Refractory Diffuse Large B-Cell Lymphoma (DLBCL): A Post-Hoc Analysis of the SADAL Study
Lead author: Josee Zijlstra, Amsterdam Universitair Medische Centra, Vrije Universiteit, Cancer Center
Abstract #: EP1226
Session: 19. Aggressive Non-Hodgkin lymphoma – Clinical

Title: Effect of Prior Therapy on the Efficacy and Safety Of Oral Selinexor in Patients With Relapsed/Refractory (R/R) Diffuse Large B-cell Lymphoma (DLBCL): A Post-hoc Analysis of the SADAL Study
Lead author: George Follows, Addenbrooke’s Hospital, Cambridge, United Kingdom
Abstract #: EP1244
Session: 19. Aggressive Non-Hodgkin lymphoma – Clinical

Title: Combined Inhibition of XPO1 and eIF4E Prevents Protein Translation resulting in Synergistic Anti-Myeloma Effects
Lead author: Shirong Li, Columbia University
Abstract #: EP1910
Session: 13. Myeloma and other monoclonal gammopathies – Biology & Translational Research

Title: IDH2 p.R172K Mutations in Patients with Acute Myeloid Leukemia (AML) May Be Associated with Favorable Response to Selinexor Treatment
Lead author: Christopher Walker, Karyopharm Therapeutics Inc.
Abstract #: EP484
Session: 03. Acute myeloid leukemia – Biology & Translational Research

Title: Comprehensive Assessment of Molecular Markers of Selinexor Response in Patients with Diffuse Large B-cell Lymphoma (DLBCL)
Lead author: Christopher Walker, Karyopharm Therapeutics Inc.
Abstract #: EP1328
Session: 20. Lymphoma Biology & Translational Research

Eltanexor

Title: Continuous XPO1 Inhibition with Eltanexor is Highly Effective in NPM1-mutated AML In Vivo
Lead author: Giulia Pianigiani, University of Perugia
Abstract #: EP441
Session: 03. Acute myeloid leukemia – Biology & Translational Research

Title: RNA and DNA Sequencing Reveal Markers of Response to the XPO1 Inhibitor Eltanexor in Patients with Relapsed or Refractory Multiple Myeloma (RRMM)
Lead author: Christopher Walker, Karyopharm Therapeutics Inc.
Abstract #: EP890
Session: 13. Myeloma and other monoclonal gammopathies – Biology & Translational Research

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. Karyopharm received 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 supplemental New Drug Application was accepted by the FDA seeking accelerated approval for selinexor as a new treatment for patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL), and selinexor has received Fast Track and Orphan designation and Priority Review from the FDA with a scheduled PDUFA date of June 23, 2020 for this patient population. 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), for which Karyopharm announced positive top-line results in March 2020. In May 2020, Karyopharm submitted a supplemental New Drug Application based on data from the Phase 3 BOSTON study. Additional, ongoing trials for selinexor include as a potential backbone therapy in combination with approved myeloma 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.

For more information about Karyopharm’s products or clinical trials, please contact the Medical Information department at:

Tel: +1 (888) 209-9326
Email: [email protected]

IMPORTANT SAFETY INFORMATION

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
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.

About Eltanexor (KPT-8602)

Eltanexor (KPT-8602) is a second generation oral SINE compound, which is currently being investigated in clinical trials. Eltanexor functions by binding to and inhibiting the nuclear export protein XPO1 (also called CRM1), leading to the accumulation of tumor suppressor proteins in the cell nucleus. Eltanexor has demonstrated minimal brain penetration in animals, which has been associated with reduced toxicities in preclinical studies while maintaining potent anti-tumor effects.

TG Therapeutics Announces Data Presentations at the 25th European Hematology Association (EHA) Annual Congress

On June 12, 2020 TG Therapeutics, Inc. (NASDAQ: TGTX), reported data presentations at the 25th European Hematology Association (EHA) (Free EHA Whitepaper) annual congress including data from a Phase 1 study evaluating TG-1701, the Company’s once daily, selective, BTK inhibitor, as monotherapy and in combination with umbralisib and ublituximab (U2) in relapsed/refractory chronic lymphocytic leukemia (CLL) and lymphoma, as well as long term data from a Phase 1/1b study evaluating the combination of umbralisib and ibrutinib in relapsed/refractory CLL and mantle cell lymphoma (MCL) (Press release, TG Therapeutics, JUN 12, 2020, View Source [SID1234561032]).

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Michael S. Weiss, the Company’s Executive Chairman and Chief Executive Officer stated, "We have long been excited about the potential for dual BCR blockade by targeting both PI3K-delta and BTK in the treatment of hematologic malignancies, and these data presentations offer insight into the therapeutic potential for this dual targeted approach. We are extremely pleased to see that TG-1701 continues to exhibit an encouraging safety and efficacy profile, both as a monotherapy and in our proprietary triplet combination with U2, with additional patients now treated and with longer follow-up. We now have patients on TG-1701 for upwards of 1.5 years, with no patients having discontinued therapy due to toxicity and responses deepening over time. We were also excited to see long-term data for the all-oral combination of umbralisib and ibrutinib, which similarly demonstrated continued improvement in overall response rates, and importantly identified no long-term safety signals at over 3.5 years of follow-up, underscoring the potential combinability of umbralisib with BTK therapy." Mr. Weiss continued, "In striving towards our goal of developing novel combination treatments for patients with unmet medical needs, we are highly encouraged by the data presented today and look forward to continuing dose escalation for our proprietary triple combination of ublituximab, umbralisib and TG-1701."

Details of the data presentations are included below.

Presentation Title: Safety and activity of the once daily selective bruton tyrosine kinase (BTK) inhibitor TG-1701 in patients with chronic lymphocytic leukemia (CLL) and lymphoma

This presentation includes interim data from a Phase 1 parallel dose-escalation study of TG-1701 monotherapy and TG-1701 in combination with U2 in 82 patients with relapsed/refractory B-cell malignancies. Sixty-nine patients were treated with single agent TG-1701, of which 25 patients were treated in the monotherapy dose escalation portion of the study and received TG-1701 at doses that ranged from 100mg to 400mg once daily, and 44 patients were treated with 200mg of TG-1701 in the monotherapy dose expansion cohort. An additional 13 patients were treated in the TG-1701 plus U2 dose escalation portion of the study.

Safety and efficacy highlights include:

TG-1701 monotherapy exhibited an encouraging preliminary safety profile across all dose levels evaluated with only 3% (2/69) of patients having a dose reduction due to treatment-related adverse events (AEs), with no treatment discontinuations due to AEs in the monotherapy cohorts
In the monotherapy dose escalation cohort (n=25), TG-1701 produced partial responses at all dose levels evaluated (100mg to 400mg once daily) in CLL, MCL, Waldenström’s macroglobulinemia (WM), and small lymphocytic lymphoma (SLL)
In the monotherapy dose expansion cohort in which TG-1701 was administered at 200mg, 25 patients were evaluable for efficacy with a 92% overall response rate (ORR) observed in CLL patients (n=12), a 33% ORR in MCL patients (n=6), and a 86% ORR in WM patients (n=7)
The combination of TG-1701 plus U2 has been well tolerated and demonstrated encouraging clinical activity with a 77% ORR across all disease types (n=13), including complete responses in three patients; dose escalation continues
Presentation Title: Long term results of a Phase I/Ib study of ibrutinib in combination with umbralisib in patients with relapsed/refractory CLL or MCL

This presentation includes updated long term data from a Phase 1/1b study of patients with relapsed or refractory CLL or MCL treated with umbralisib in combination with ibrutinib. Data from this trial were previously published in Lancet Haematology in December 2018 (Davids et.al.). As of the updated data cutoff, 42 patients were evaluable for safety and efficacy (21 CLL patients and 21 MCL patients).

Safety and efficacy highlights include:

With long term follow up (median follow-up of 43.5 months (range 8.4-61), there were no cumulative or recurrent late onset toxicities observed
In relapsed/refractory CLL, the overall response rate was 95% including a 29% complete response (CR) rate, and the 4-year Progression-free Survival (PFS) and Overall Survival (OS) were 78% and 90%, respectively
In relapsed/refractory MCL, the ORR was 71% with a 24% CR rate, and median PFS and OS were 10.8 and 30.7 months, respectively
The data presented is available on the Publications page, located within the Pipeline section, of the Company’s website at www.tgtherapeutics.com/publications.cfm.