New long-term data on Calquence presented at ASH 2018

On December 3, 2018 AstraZeneca and Acerta Pharma, its haematology research and development centre of excellence, have presented new, long-term follow-up results for Calquence (acalabrutinib) in patients with relapsed or refractory mantle cell lymphoma (MCL), and updated results of an ongoing clinical trial assessing Calquence monotherapy in treatment-naïve patients with chronic lymphocytic leukaemia (CLL), at the 60th American Society of Hematology (ASH) (Free ASH Whitepaper) Annual Meeting & Exposition in San Diego, CA, USA (Press release, AstraZeneca, DEC 3, 2018, View Source [SID1234531822]).

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Sean Bohen, Executive Vice President, Global Medicines Development and Chief Medical Officer, said: "The data from these two clinical trials validate previous findings and add to the growing body of evidence that support the promise of Calquence in multiple blood cancers. We are very encouraged by these results, which reinforce our commitment to advancing innovative treatments for blood cancer patients."

Calquence follow-up data in MCL confirms efficacy and tolerability

Long-term follow-up data presented from the Phase II ACE-LY-004 trial in relapsed or refractory MCL showed sustained and clinically meaningful responses to Calquence with a median follow-up of more than two years (26 months), confirming its efficacy and safety profile in this patient population.1 Initial data from this trial served as the basis for the accelerated approval of Calquence by the US Food and Drug Administration in October 2017 and the first approval outside the US in November 2018 in the United Arab Emirates.3,4

Michael L. Wang, MD, Professor, Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center, and Principal Investigator of the ACE-LY-004 MCL trial, said: "It’s encouraging to see the sustained duration of response in the updated analysis and the safety profile of acalabrutinib maintained consistently over time in MCL patients. As we gain more and more experience with this therapy, its importance as a treatment option for relapsed or refractory MCL is being more fully realised across the clinical and patient community."

Summary of key investigator-assesseda efficacy results from the open-label, single-arm clinical trial of Calquence in 124 adult patients with relapsed or refractory MCL1:

Efficacy measure Result (N=124; 95% CIb)
Overall response rate

(Complete response + partial response)

81% (73, 87)

Best response

Complete response

43% (34, 52)

Partial response

38% (29, 47)

Stable disease

9% (5, 15)

Progressive disease

8% (4, 14)

Not evaluablec

2% (1, 7)

Median duration of response

26 months (17.5, not reached)

Median progression-free survival

20 months (16.5, 27.7)

a Response was assessed based on the Lugano classification.

b Confidence interval (CI).

c Includes patients without any adequate post-baseline disease assessment.

The median follow-up was 26 months, with 40% of patients remaining on treatment with Calquence at the time of analysis. An exploratory analysis of the trial found that an undetectable minimal residual disease status was achieved in a sub-set of patients.

In this trial, the most frequent adverse events (AEs; ≥ 20%, all grades) were headache (38%), diarrhoea (36%), fatigue (28%), cough (22%) and myalgia (21%). These events were primarily Grade 1/2. Grade 3/4 AEs (≥ 5%) were anaemia (11%), neutropoenia (11%) and pneumonia (6%). There were 13 patients (10%) with 16 cardiac events including four Grade 3/4 events, each in one patient (acute coronary syndrome, acute myocardial infarction, cardiorespiratory arrest, coronary artery disease). There was no new onset of atrial fibrillation. Bleeding events occurred in 33% of patients, most frequently contusion (13%) and all bleeding events but three (2%, Grade 3) were Grade 1/2 events. Ten patients discontinued treatment due to AEs. In total there were six deaths due to AEs (none of which were considered to be related to Calquence).1

New data from ongoing CLL clinical trial demonstrate strong efficacy

Updated results of the Phase I/II ACE-CL-001 trial were presented today in an oral session. In a cohort of treatment-naïve patients with CLL, long-term safety and efficacy of assessment showed high response rates with no new safety signals identified. The median time on trial was 42 months, with 89% of patients remaining on treatment with Calquence at the time of analysis.2

John C. Byrd, MD, Distinguished University Professor, The Ohio State University, and Principal Investigator for the ACE-CL-001 CLL clinical trial, said: "A key challenge in the treatment of CLL is ensuring patients have therapies that they can tolerate and benefit from over the long term. The results seen in this patient cohort at 3.5 years of follow-up are encouraging for both durability of response and tolerability of therapy. We look forward to continued data from ongoing studies evaluating acalabrutinib in CLL."

Summary of key investigator-assesseda efficacy results from the Phase I/II open-label, single-arm ACE-CL-001 Calquence trial in 99 patients with CLL, evaluating the treatment-naïve cohort2:

Efficacy measure Result (N=99)
Overall response rate

(Complete response + partial response)

97%

Complete response

5%

Partial response

92%

Median duration of responseb

NR (range, 42.4 to NR)c

36 month duration of response rate (95% CI)b,d,e

98% (90, 99)

Median progression-free survival

NR (range, 44.2 to NR)c

36 month progression-free survival rate (95% CI)d,e

97% (91, 99)

a Response was assessed using International Workshop on Chronic Lymphocytic Leukemia (IWCLL) 2008 criteria with modification for lymphocytosis.

b Only responders with ≥ partial response were included in this analysis.

c Not reached (NR).

d Confidence interval (CI).

e Based on the Kaplan-Meier estimates.

In this trial, the most common AEs (≥ 20%, all grades) were diarrhoea (49%), headache (44%), upper respiratory tract infection (40%), contusion (39%), arthralgia (33%), weight increased (31%), nausea (30%) and cough (23%). Grade 3/4 AEs (≥ 5%) were neutropenia (8%), hypertension (7%), diarrhoea (5%) and headache (5%). Atrial fibrillation and hypertension (all grades) occurred in 6% and 17% of patients, respectively, with Grade 3 events occurring in 2% and 7% of patients. Bleeding events (all grades) occurred in 64% of patients with contusion being most common (39%). All but three (3% Grade 3) bleeding events were Grade 1/2 events and no patients discontinued due to bleeding. Overall, 11% of patients discontinued treatment, 5% of which were due to AEs, including secondary malignancies (angiosarcoma, glioblastoma multiforme, small cell lung cancer), sepsis (Grade 4) and urinary tract infection (Grade 3). One Grade 5 event (multiorgan failure) in the setting of pneumonia was reported, which was considered unrelated to Calquence.2

NOTES TO EDITORS
About Calquence

Calquence (acalabrutinib) is an inhibitor of Bruton tyrosine kinase (BTK). Calquence binds covalently to BTK, thereby inhibiting its activity.3 In B cells, BTK signalling results in activation of pathways necessary for B cell proliferation, trafficking, chemotaxis, and adhesion.3

Calquence was granted accelerated approval by the US Food and Drug Administration (FDA) in October 2017 for the treatment of adult patients with mantle cell lymphoma (MCL) who have received at least one prior therapy. Continued approval for this indication may be contingent upon verification and description of clinical benefit in confirmatory trials. Calquence is not currently licensed for the treatment of chronic lymphocytic leukaemia (CLL).

Calquence was granted Orphan Drug designation by the European Commission in 2016 and the US FDA in 2015 for the treatment of patients with CLL, MCL and Waldenstrom’s macroglobulinemia; and Breakthrough Therapy Designation in August 2017 by the US FDA for the treatment of patients with MCL who have received at least one prior therapy.

About mantle cell lymphoma (MCL)

MCL is an uncommon type of B-cell non-Hodgkin lymphoma.5,6,7 MCL comprises 3% to 6% of non-Hodgkin lymphomas, with an annual incidence of 0.5 per 100,000 population in Western countries; in the US, it was estimated that approximately 3,300 new cases of MCL were diagnosed in 2016.5,8 The median age at diagnosis is 68 years,5 with MCL occurring more than twice as often in men than women.7 While MCL patients initially respond to treatment, there is a high relapse rate.5

About chronic lymphocytic leukaemia (CLL)

CLL is the most common type of leukaemia in adults and accounts for approximately one in four cases of leukaemia.9,10 The average age at the time of diagnosis is approximately 70 years of age.10 In CLL, too many blood stem cells in the bone marrow become abnormal lymphocytes and these abnormal cells have difficulty fighting infections.9 As the number of abnormal cells grows there is less room for healthy white blood cells, red blood cells and platelets.9 This could result in anaemia, infection and bleeding.9 B cell receptor signalling through BTK is one of the essential growth pathways for CLL

NeoImmuneTech Presents Hyleukin-7 and Universal CAR-T Combination Therapy Preclinical Data at ASH Annual Meeting

On December 3, 2018 NeoImmuneTech, Inc., an immunotherapy drug development company focused on advanced cancer treatments, reported the presentation of two preclinical studies supporting the potential of combining Hyleukin-7 with Universal CAR-T (UCART) treatment to enhance and prolong the activity of allogeneic "off-the-shelf" adoptive immunotherapy for the treatment of B cell and T cell lymphomas (Press release, NeoImmuneTech, DEC 3, 2018, View Source [SID1234531839]). The data were presented in an oral presentation and a poster session at the 60th American Society of Hematology (ASH) (Free ASH Whitepaper) Annual Meeting and Exposition in San Diego, CA.

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The two studies, conducted in collaboration with researchers from Washington University in St. Louis, evaluated the effects that Hyleukin-7, a long-acting T cell amplifier, had in combination with different types of UCART treatments. The researchers evaluated Hyleukin-7’s effects on proliferation, differentiation and tumor killing capacity of UCARTs in tumor models of B cell lymphoma and T cell lymphoma. The T cell lymphoma model was a physiologically-relevant patient-derived xenograft (PDX) mouse model of Sézary syndrome (SS), an aggressive and highly-morbid cutaneous T cell lymphoma with no widely-effective treatments.

Hyleukin-7 was shown to dramatically enhance UCART proliferation, persistence and tumor killing in vivo, resulting in enhanced survival in both models. Furthermore, the results suggest that the addition of Hyleukin-7 dramatically increased the activity of UCART treatment and could provide a tunable, clinic-ready "enabling therapy" for suboptimal CART activity in vivo.

"NeoImmuneTech’s Hyleukin-7 has broad potential to strengthen our immune system in its battle against both foreign invaders as well as enemies within, adding a therapeutic effect wherever the body’s T cells are weak or in low numbers," said Se Hwan Yang, Ph.D., Chief Executive Officer of NeoImmuneTech. "These latest experiments in lymphoma models show that Hyleukin-7 may also strengthen and prolong the effects of T cell therapies, such as CAR-T. The beneficial effects of Hyleukin-7 when combined with UCART are highly encouraging, as developing an effective, off-the-shelf and universal CAR-T therapeutic is a major goal for immuno-oncology drug developers. NeoImmuneTech’s main focus is advancing our clinical trials with Hyleukin-7 as efficiently as possible so that we can ultimately deliver it in a timely manner to patients in need."

Dr. John F. DiPersio, M.D., Ph.D., Chief, Division of Oncology and Deputy Director of the Alvin J. Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine added: "CAR-T therapies hold a great promise for cancer patients, as well as for other diseases. We have made incredible steps toward creating a universal CAR-T that would highly increase the percent of people that can benefit from this technology, while significantly decreasing the time to treatment and the cost of production. However, a major hurdle that still remains for both current and future CAR-T treatments is the short effective period for these cells in the body. Hyleukin-7 has the potential to boost both the time and the effectivity of CAR-T treatments, and we are looking forward to the continued clinical development of this drug candidate."

Presentation Details:
Abstract number: 340
Title: Modeling Sézary Syndrome for Immunophenotyping and Anti-Tumor Effect of Ucart and Long-Acting Interleukin-7 Combination Therapy
Type: Oral presentation
Time: Sunday, December 2, 2018: 10:15 AM
Location: Room 28D (San Diego Convention Center)

Abstract number: 2199
Title: A Long-Acting Pharmacological Grade Interleukin-7 Molecule Logarithmically Accelerates Ucart Proliferation, Differentiation, and Tumor Killing
Type: Poster presentation
Time: Saturday, December 1, 2018, 6:15 PM-8:15 PM
Location: Hall GH (San Diego Convention Center)

About Hyleukin-7
Hyleukin-7 (rhIL-7-hyFc, NT-I7), an immuno-oncology agent, is a T cell amplifier comprising a covalently linked homodimer of engineered Interleukin-7 (IL-7) molecule, biologically fused with the proprietary long-acting platform – hyFc. IL-7 is known to be a critical factor for T cells, acting to increase both the number and functionality of T cells. Hyleukin-7 could play a pivotal role in reconstituting and reinvigorating T cell immunity in the treatment of patients with cancer and lymphopenia, as well as providing unique opportunities for immuno-oncology (IO) combination strategies. Hyleukin-7 is being developed as an "IO enabling" therapy to harness T cell immunity in combination with current cancer treatments such as anti-PD-(L)1 agents or chemo/radiotherapy. NeoImmuneTech and Genexine, Inc. (Genexine) are collaborating in three Phase 1b/2a clinical trials in advanced solid tumors and glioblastoma in the US and Korea.

Innate Pharma announces updated results that support advancement of IPH4102 in refractory Sézary syndrome at the American Society of Hematology (ASH) 2018 annual meeting

On December 3, 2018 Innate Pharma SA (the "Company" – Euronext Paris: FR0010331421 – IPH), reported updated data from the Phase I trial (including an expansion cohort) evaluating IPH4102 in refractory patients with Sézary syndrome (SS) and its plan to advance IPH4102 in a multi-cohort Phase II study in different subtypes of T-cell lymphoma (Press release, Innate Pharma, DEC 3, 2018, View Source [SID1234531855]). An oral presentation will take place on Monday, December 3, at the ASH (Free ASH Whitepaper) 2018 Annual Meeting in San Diego, USA, by Pr Martine Bagot, Head of the Dermatology Department at the Saint Louis Hospital, Paris and Principal Investigator of the study. IPH4102 is Innate Pharma’s wholly-owned first-in-class anti-KIR3DL2 antibody, designed for treatment of T-cell lymphoma.

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As of October 15, 2018, data from the subgroup of 35 SS patients revealed strong clinical activity, demonstrated by an overall response rate (ORR) of 42.9%, median duration of response (DoR) of 13.8 months and median progression-free survival (PFS) of 11.7 months.

Mogamulizumab pretreated patients (n=7) showed an ORR (42.9%), median DoR (13.8 months) and median PFS (16.8 months) similar to the entire group. The ORR appeared to be higher (n = 28, 53.6%) in patients with no histologic evidence of large cell transformation (LCT*).

"The solid updated data on IPH4102 presented today strongly encourage us to advance IPH4102 in refractory Sézary syndrome patients. We believe that the planned Phase II study, together with the Phase I data, has the potential to support a BLA submission in this indication," commented Pierre Dodion, Chief Medical Officer of Innate Pharma. "In addition, the expression profile of KIR3DL2 provides a strong rational to explore the potential of IPH4102 in other subtypes of T-cell lymphomas in the TELLOMAK phase II study".

Importantly, clinical activity was associated with a substantial improvement in quality of life as assessed by the SkinDex29 and Pruritus Visual Analog Scale (VAS) scores. IPH4102 displayed a favorable safety profile, consistent with previous observations.

"Refractory Sézary syndrome patients have limited effective treatment options in later lines of therapy, with toxicity remaining an area of great concern with currently approved drugs," commented Professor Martine Bagot, Principal Investigator of the study. "IPH4102, in addition to demonstrating an impressive clinical activity, has shown a favorable safety profile and substantially improves the quality of life even in patients with stable disease. Translational results show relevant pharmacodynamics effects of IPH4102 in skin and in blood, which are in line with the clinical efficacy of the drug."

Exploratory biomarker analysis show early elimination of aberrant tumor cells and peripheral blood KIR3DL2+ CD4 T cells upon IPH4102 administration in responding patients.

The presentation is available in the IPH4102 section on Innate Pharma’s website.

KOL webcast and conference call on Tuesday, December 4, at 5pm CET (8am PST)

Pierre Dodion, Chief Medical Officer, Innate Pharma, will be joined by Prof. Martine Bagot, Head of Dermatology Department at the Saint Louis Hospital, Paris and lead investigator of the study, for a live webcast and conference call with a Q&A session on Tuesday, December 4 at 5pm CET to discuss the announcement.

The presentation and access to the live webcast will be available at this link: View Source

Participants can also join the conference call using the following dial-in numbers:

Location

Purpose

Phone number

France

Participant

+33 (0)1 76 77 22 57

United Kingdom

Participant

+44 (0)330 336 9411

United States

Participant

+1 929-477-0324

Standard International Access

Participant

0800 279 7204

The participation code is: 4535688

Agios Presents Updated Data from the Ivosidenib Phase 1 Dose-Escalation and Expansion Trial in IDH1 Mutant Positive Patients with Newly Diagnosed Acute Myeloid Leukemia (AML) Ineligible for Standard Treatment and Myelodysplastic Syndrome (MDS)

On December 3, 2018 Agios Pharmaceuticals, Inc. (NASDAQ:AGIO), a leader in the field of cellular metabolism to treat cancer and rare genetic diseases, reported its updated efficacy and safety data from the untreated acute myeloid leukemia (AML) arm from the ongoing Phase 1 dose-escalation and expansion study evaluating single agent ivosidenib in patients with hematologic malignancies and an isocitrate dehydrogenase-1 (IDH1) mutation (Press release, Agios Pharmaceuticals, DEC 3, 2018, View Source [SID1234531872]). The data were featured in an oral presentation at the 60thAmerican Society of Hematology Annual Meeting in San Diego.

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"Ivosidenib induced deep, durable remissions in newly diagnosed AML patients who are older, have high rates of secondary AML and prior hypomethylating agent exposure," said Gail Roboz, M.D., Weill Cornell Medical College and an investigator in the study. "Ivosidenib had a favorable safety profile characterized by a low rate of febrile neutropenia and infections. In addition, transfusion independence was observed across response categories, including in patients who did not achieve complete remission."

"We believe these data are encouraging and represent compelling evidence for the potential of single agent ivosidenib as a new treatment option for newly diagnosed AML patients who are ineligible for standard therapies," said Chris Bowden, M.D., chief medical officer at Agios. "There is tremendous need for targeted treatment options for these patients who are typically older and have comorbid conditions, and we are on track to submit a supplemental new drug application for ivosidenib for this patient population under the FDA Real Time Oncology Review pilot program by the end of January 2019."

Untreated AML Data Presentation
As of the May 11, 2018 data cutoff, a total of 258 patients with advanced hematologic malignances and an IDH1 mutation were treated in the Phase 1 study, including 34 patients with untreated AML (nine from dose-escalation and 25 from expansion) who received 500 mg of ivosidenib daily. Enrollment to the study is closed.

Among the untreated AML patients, 20.6% had de novo AML and 79.4% had secondary AML (sAML).
The median age for these patients was 76.5 years (64-87) and 41.2% had received a prior hypomethylating agent.
The median treatment duration for the untreated AML patients was 4.3 months (0.3-35.1).
Safety Data
As of the data cut-off, a safety analysis conducted for the 34 untreated AML patients showed that ivosidenib demonstrates a safety profile that is consistent with previously reported data for all 258 patients. The most common adverse events (AEs) of any grade >25% regardless of causality were diarrhea (52.9%), fatigue (44.1%), nausea (38.2%), decreased appetite (32.4%), leukocytosis (26.5%), anemia (26.5%) and edema peripheral (26.5%). Adverse events of interest were the following:

8.8% reported Grade ≥3 ECG QT prolongation. Ivosidenib was dose reduced in two patients and held in four patients (for any grade of ECG QT prolongation).
17.6% reported IDH-differentiation syndrome (IDH-DS) of any grade, which was managed with corticosteroids and diuretics. Three patients had their dose temporarily held, and no patients required dose reductions.
3% reported Grade ≥3 leukocytosis.
No AEs of interest lead to any permanent treatment discontinuations or deaths.
Efficacy Data
Data from 33 untreated AML patients with an IDH1 mutation confirmed by the Abbott RealTime IDH1 assay demonstrated an overall response rate (ORR) of 57.6% (19 of 33 patients) [95% CI 39.2, 74.5] and a combined complete remission (CR) and CR with partial hematologic recovery (CRh) rate of 42.4% [95% CI 25.5, 60.8] which is the primary endpoint of the study.

The CR rate was 30.3% (10 of 33 patients) [95% CI 15.6, 48.7] and the CRh rate was 12.1% (4 of 33 patients) [95% CI 3.4, 28.2]. CRh is defined as <5% of blasts in the bone marrow, no evidence of disease and partial recovery of peripheral blood counts (ANC >500/microliter and platelets >50,000/microliter).
Median time to first response was 1.9 months (range 0.9, 3.6) for all patients who responded and median time to CR/CRh was 2.8 months (range 1.9, 12.9).
Median durations of CR, CR+CRh, and ORR were not estimable (lower bound of 95% CI 4.2, 6.5 and 6.5 months, respectively); the estimated 12-month durations of response were 77.8%, 66.7% and 59.5%, respectively.
Transfusion independence, defined as an absence of transfusions for at least 56 consecutive days on treatment, was observed across all response categories.
Of the patients who achieved a CR or CRh and were transfusion dependent at baseline, all became independent of platelet and RBC transfusions during any 56-day post baseline period.
Achievement of transfusion independence was also seen among some non-CR/CRh responders and non-responders.
IDH1 mutation clearance, defined as a reduction in mIDH1 variant allele frequency to below the limit of detection of 0.02–0.04% (2-4 x10-4), was observed in 64% (9/14) of patients with untreated AML who achieved CR or CRh, including 50% (5/10) of patients with CR and 100% (4/4) of patients with CRh.
MDS Data Presentation
Updated safety and efficacy data based on May 11, 2018 data cutoff were also presented on December 1, 2018 for 12 myelodysplastic syndrome (MDS) patients from the dose-escalation (n=3) and expansion (n=9) portions of the Phase 1 study whose starting dose was 500 mg daily. The median age was 72.5 years (52-78).

The most common AEs of any grade occurring in ≥20% of patients were back pain and fatigue (n=4, 33.3% each) and anemia, decreased appetite, diarrhea, dyspnea, hypokalemia, pruritus, and rash (n=3, 25% each). Most AEs were grade 1–2 and reported as unrelated to treatment. No AEs led to permanent discontinuation of treatment.
Grade 2 IDH differentiation syndrome (IDH-DS) was observed in 1 of 12 patients.
Of the 12 patients with MDS, five achieved CR (41.7%) [95% CI (15.2%, 72.3%)], one achieved a partial response (PR) (8.3%) and five achieved marrow CR (mCR) (41.7%), resulting in an ORR of 91.7% [95% CI (61.5%, 99.8%)].
The median durations of CR was not estimable at the time of the data cutoff; the median duration of response was 21.4 months with 95% CI [2.3, NE]. The percentages of patients who remained in CR and response at 12 months were 60.0% and 61.4%, respectively.
Among the five patients who were transfusion dependent at baseline, four became transfusion independent for at least 56 days on treatment.
TIBSOVO (ivosidenib) is not approved for the treatment of patients with newly diagnosed AML or MDS by any regulatory authority.

About TIBSOVO (ivosidenib)

TIBSOVO (ivosidenib) is an isocitrate dehydrogenase-1 (IDH1) inhibitor indicated for the treatment of adult patients with relapsed or refractory acute myeloid leukemia (AML) with a susceptible IDH1 mutation as detected by an FDA-approved test. For more information, visit TIBSOVO.com.

IMPORTANT SAFETY INFORMATION

WARNING: DIFFERENTIATION SYNDROME

Patients treated with TIBSOVO have experienced symptoms of differentiation syndrome, which can be fatal if not treated. Symptoms may include fever, dyspnea, hypoxia, pulmonary infiltrates, pleural or pericardial effusions, rapid weight gain or peripheral edema, hypotension, and hepatic, renal, or multi-organ dysfunction. If differentiation syndrome is suspected, initiate corticosteroid therapy and hemodynamic monitoring until symptom resolution.
WARNINGS AND PRECAUTIONS

Differentiation Syndrome: See Boxed WARNING. In the clinical trial, 19% (34/179) of patients with relapsed or refractory AML treated with TIBSOVO experienced differentiation syndrome. Differentiation syndrome is associated with rapid proliferation and differentiation of myeloid cells and may be life-threatening or fatal if not treated. Symptoms of differentiation syndrome in patients treated with TIBSOVO included noninfectious leukocytosis, peripheral edema, pyrexia, dyspnea, pleural effusion, hypotension, hypoxia, pulmonary edema, pneumonitis, pericardial effusion, rash, fluid overload, tumor lysis syndrome, and creatinine increased. Of the 34 patients who experienced differentiation syndrome, 27 (79%) recovered after treatment or after dose interruption of TIBSOVO. Differentiation syndrome occurred as early as 1 day and up to 3 months after TIBSOVO initiation and has been observed with or without concomitant leukocytosis.

If differentiation syndrome is suspected, initiate dexamethasone 10 mg IV every 12 hours (or an equivalent dose of an alternative oral or IV corticosteroid) and hemodynamic monitoring until improvement. If concomitant noninfectious leukocytosis is observed, initiate treatment with hydroxyurea or leukapheresis, as clinically indicated. Taper corticosteroids and hydroxyurea after resolution of symptoms and administer corticosteroids for a minimum of 3 days. Symptoms of differentiation syndrome may recur with premature discontinuation of corticosteroid and/or hydroxyurea treatment. If severe signs and/or symptoms persist for more than 48 hours after initiation of corticosteroids, interrupt TIBSOVO until signs and symptoms are no longer severe.

QTc Interval Prolongation: Patients treated with TIBSOVO can develop QT (QTc) prolongation and ventricular arrhythmias. One patient developed ventricular fibrillation attributed to TIBSOVO. Concomitant use of TIBSOVO with drugs known to prolong the QTc interval (e.g., anti-arrhythmic medicines, fluoroquinolones, triazole anti-fungals, 5-HT3 receptor antagonists) and CYP3A4 inhibitors may increase the risk of QTc interval prolongation. Conduct monitoring of electrocardiograms (ECGs) and electrolytes. In patients with congenital long QTc syndrome, congestive heart failure, electrolyte abnormalities, or in those who are taking medications known to prolong the QTc interval, more frequent monitoring may be necessary.

Interrupt TIBSOVO if QTc increases to greater than 480 msec and less than 500 msec. Interrupt and reduce TIBSOVO if QTc increases to greater than 500 msec. Permanently discontinue TIBSOVO in patients who develop QTc interval prolongation with signs or symptoms of life-threatening arrhythmia.

Guillain-Barré Syndrome: Guillain-Barré syndrome occurred in <1% (2/258) of patients treated with TIBSOVO in the clinical study. Monitor patients taking TIBSOVO for onset of new signs or symptoms of motor and/or sensory neuropathy such as unilateral or bilateral weakness, sensory alterations, paresthesias, or difficulty breathing. Permanently discontinue TIBSOVO in patients who are diagnosed with Guillain-Barré syndrome.

ADVERSE REACTIONS

The most common adverse reactions (≥20%) of any grade were fatigue (39%), leukocytosis (38%), arthralgia (36%), diarrhea (34%), dyspnea (33%), edema (32%), nausea (31%), mucositis (28%), electrocardiogram QT prolonged (26%), rash (26%), pyrexia (23%), cough (22%), and constipation (20%).
The most frequently reported ≥Grade 3 adverse reactions (≥5%) were electrocardiogram QT prolonged (10%), dyspnea (9%), leukocytosis (8%), tumor lysis syndrome (6%), and differentiation syndrome (5%).
Serious adverse reactions (≥5%) were differentiation syndrome (10%), leukocytosis (10%), and electrocardiogram QT prolonged (7%). There was one case of progressive multifocal leukoencephalopathy (PML).
DRUG INTERACTIONS

Strong or Moderate CYP3A4 Inhibitors: Reduce TIBSOVO dose with strong CYP3A4 inhibitors. Monitor patients for increased risk of QTc interval prolongation.

Strong CYP3A4 Inducers: Avoid concomitant use with TIBSOVO.

Sensitive CYP3A4 Substrates: Avoid concomitant use with TIBSOVO.

QTc Prolonging Drugs: Avoid concomitant use with TIBSOVO. If co-administration is unavoidable, monitor patients for increased risk of QTc interval prolongation.

LACTATION

Many drugs are excreted in human milk and because of the potential for adverse reactions in breastfed children, advise women not to breastfeed during treatment with TIBSOVO and for at least 1 month after the last dose.

Please see full Prescribing Information, including Boxed WARNING.

About Acute Myelogenous Leukemia (AML)
AML, a cancer of blood and bone marrow characterized by rapid disease progression, is the most common acute leukemia affecting adults. Undifferentiated blast cells proliferate in the bone marrow rather than mature into normal blood cells. AML incidence significantly increases with age, and the median age of diagnosis is 68. The vast majority of patients do not respond to chemotherapy and progress to relapsed/refractory AML. The five-year survival rate for AML is approximately 27 percent. IDH1 mutations are present in about 6 to 10 percent of AML cases.

About Myelodysplastic Syndrome (MDS)
MDS comprises a diverse group of bone marrow disorders in which immature blood cells in the bone marrow do not mature or become healthy blood cells. The National Cancer Institute estimates that more than 10,000 people are diagnosed with MDS in the U.S. each year. Failure of the bone marrow to produce mature healthy cells is a gradual process, and reduced blood cell and/or reduced platelet counts may be accompanied by the loss of the body’s ability to fight infections and control bleeding. For roughly 30 percent of the patients diagnosed with MDS, this bone marrow failure will progress to AML. Chemotherapy and supportive blood products are used to treat MDS.

Aprea Therapeutics Closes EUR 50 Million Financing

On December 2, 2018 Aprea Therapeutics, a clinical-stage biotechnology company developing novel anticancer therapies targeting the p53 tumor suppressor protein, reported the closing of a EUR 50 million Series C financing round led by the Redmile Group, with participation by new investor Rock Springs Capital and existing investors 5AM Ventures, Versant Ventures, HealthCap, Sectoral Asset Management and Karolinska Development AB (Nasdaq Stockholm: KDEV) (Press release, Aprea, DEC 3, 2018, View Source [SID1234531806]). A representative from Redmile Group will also be appointed as Director to the Aprea Therapeutics Board of Directors.

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"We are thrilled to close this financing, initiate a Phase III clinical study in myelodysplastic syndromes (MDS) with our lead compound, APR-246, and drive the company toward its next value inflection point," said Christian S. Schade, President and Chief Executive Officer of Aprea. "The addition of Redmile and Rock Springs to our investor group broadens the US investor base and we believe positions the company for future strategic opportunities. We are committed to bringing this important new therapy to cancer patients with limited therapeutic options."

Proceeds from the financing will be used to advance the clinical development of APR-246, a first-in-class anticancer agent that reactivates mutated p53 tumor suppressor protein. Aprea is planning to begin a Phase 3 clinical study in myelodysplastic syndromes (MDS) and is nearing completion of a Phase Ib/II clinical trial in p53 mutated high-risk myelodysplastic syndromes (MDS) and oligoblastic acute myeloid leukemia (AML) with APR-246 and azacitidine. The Phase II part of the study is ongoing, with updated data to be presented at the 2018 American Society of Hematology (ASH) (Free ASH Whitepaper) annual meeting.

About Myelodysplastic Syndromes

Myelodysplastic syndromes (MDS) represents a spectrum of hematopoietic stem cell malignancies in which bone marrow fails to produce sufficient numbers of healthy blood cells. Approximately 30-40% of MDS patients progress to acute myeloid leukemia (AML) and mutation of the p53 tumor suppressor protein is thought to contribute to disease progression. Mutations in p53 are found in up to 20% of MDS and AML patients and are associated with poor overall prognosis.

About p53 and APR-246

The p53 tumor suppressor gene is the most frequently mutated gene in human cancer, occurring in approximately 50% of all human tumors. These mutations are often associated with resistance to anti-cancer drugs and poor overall survival, representing a major unmet medical need in the treatment of cancer.

APR-246 has been shown to reactivate mutant and inactivated p53 protein – by restoring wild-type p53 conformation and function – and thereby induce programmed cell death in human cancer cells. APR-246 has demonstrated pre-clinical anti-tumor activity in a wide variety of solid and hematological (blood) tumors, including MDS, AML, and ovarian cancer, among others. Additionally, strong synergy has been seen with both traditional anti-cancer agents, such as chemotherapy, as well as newer mechanism-based anti-cancer drugs and immuno-oncology checkpoint inhibitors. In addition to pre-clinical testing, a Phase I/II clinical program with APR-246 has been completed, demonstrating a favorable safety profile and both biological and confirmed clinical responses in hematological malignancies and solid tumors with mutations in the TP53 gene.