These results showed reduction in tumor
volume, building on the established safety and efficacy profile of
KOSELUGO in certain children and has the potential to support
expanded use in certain adults
Alexion, AstraZeneca Rare Disease and Merck (NYSE: MRK), known
as MSD outside of the United States and Canada, today announced
positive topline results from the Phase 3 KOMET trial, which is the
largest, global randomized double-blind placebo-controlled
multicenter Phase 3 trial in adults with neurofibromatosis type 1
(NF1) who have symptomatic, inoperable plexiform neurofibromas
(PN). Topline results showed that KOSELUGO, an oral, selective MEK
inhibitor, demonstrated a statistically significant and clinically
meaningful improvement in objective response rate (ORR), the
study’s primary endpoint, versus placebo, in these adult
patients.
Neurofibromatosis type 1 is a rare, progressive genetic
condition affecting an estimated 1.7 million people worldwide,
approximately 70% of whom are adults. In 30-50% of patients, tumors
develop on the nerve sheaths and may cause debilitating symptoms.
Neurofibromatosis type 1 is usually diagnosed in early childhood;
however, NF1 often progresses into adulthood. There are no approved
treatments for adults, leaving many to experience disfigurement,
dysfunction, persistent pain or endure multiple surgeries.
Professor Ignacio Blanco Guillermo, M.D., Ph.D., chairman,
genetic counseling and clinical genetics program at the Germans
Trias i Pujol University Hospital, chairman, Spanish National
Reference Center for Adult Patients with Neurofibromatosis and
principal investigator of the KOMET trial, said, “With limited
options to manage NF1 plexiform neurofibromas in adults, many
patients experience functional impairment and symptoms, which can
substantially impact their lives. These clinically meaningful data
show KOSELUGO has the potential to make a positive impact in
patient care by reducing the size of plexiform neurofibromas.”
Marc Dunoyer, chief executive officer, Alexion, said, “These
promising results demonstrate that KOSELUGO, the first and only
approved targeted therapy for certain children with NF1 plexiform
neurofibromas, now has the potential to benefit adult patients for
whom there are no approved targeted therapies. As the largest and
only global placebo-controlled Phase 3 trial in adults with NF1
plexiform neurofibromas, KOMET reinforces our leadership in
advancing potential treatment options for people living with this
debilitating disease. We look forward to sharing these findings
with regulatory authorities.”
Dr. Scot Ebbinghaus, vice president, global clinical
development, Merck Research Laboratories, said, “Adults with NF1
are in critical need of treatment options to help manage
symptomatic, inoperable plexiform neurofibromas. These positive
results from the Phase 3 KOMET trial demonstrate the potential to
expand the use of KOSELUGO beyond pediatric patients to also treat
adult patients living with this rare and challenging genetic
condition.”
In the trial, ORR was defined as the percentage of patients with
confirmed complete response (disappearance of PNs) or partial
response (at least 20% reduction in tumor volume) by cycle 16 (28
days per cycle) as determined by independent central review (ICR)
per response evaluation in neurofibromatosis and schwannomatosis
(REiNS) criteria.
The safety profile of KOSELUGO in this study was consistent with
that observed in clinical trials among children and adolescents. No
new safety signals were identified.
Alexion, AstraZeneca Rare Disease will share these data with
regulatory authorities and present these data at a forthcoming
medical meeting. AstraZeneca and Merck are jointly developing and
commercializing KOSELUGO globally.
About KOMET KOMET is a global randomized, double-blind,
placebo-controlled, multi-center Phase 3 trial (ClinicalTrials.gov,
NCT04924608) designed to evaluate the efficacy and safety of
KOSELUGO in adults with NF1 who have symptomatic, inoperable PNs.
The trial enrolled 145 adults from 13 countries across the U.S.,
Asia, Australia, South America and Europe, with participants’
baseline characteristics, including gender and distribution of PNs,
reflective of the global adult NF1 patient population. Patients
were enrolled and randomized 1:1 to receive KOSELUGO or placebo for
twelve 28-day cycles. Participants were required to have diagnosis
of NF1 at least one, symptomatic, inoperable target PN measurable
by volumetric MRI analysis, chronic PN pain score documented during
screening, adequate organ and marrow function and stable chronic PN
pain medication use at enrollment.
The primary endpoint is confirmed ORR by cycle 16 as assessed by
ICR. Objective response rate is defined as the percentage of
patients with confirmed complete response (disappearance of PNs) or
partial response (at least 20% reduction in tumor volume).
After 12 cycles, patients on placebo were switched to KOSELUGO
and patients on KOSELUGO remained on treatment for an additional 12
cycles. Patients who had the opportunity to complete both treatment
periods 24 cycles of treatment have the option to participate in a
long-term extension period and continue to receive KOSELUGO.
About NF1 Neurofibromatosis type 1 is a rare,
progressive, genetic condition that is caused by a spontaneous or
inherited mutation in the NF1 gene. Neurofibromatosis type 1 is
associated with a variety of symptoms, including soft lumps on and
under the skin (cutaneous neurofibromas) and, in 30-50% of
patients, tumors develop on the nerve sheaths (PNs). These PNs can
cause clinical issues such as disfigurement, motor dysfunction,
pain, airway dysfunction, visual impairment and bladder or bowel
dysfunction. Plexiform neurofibromas begin during early childhood,
with varying degrees of severity, and can reduce life expectancy by
up to 15 years.
About KOSELUGO® (selumetinib) KOSELUGO is a kinase
inhibitor that blocks specific enzymes (MEK1 and MEK2), which are
involved in stimulating cells to grow. In NF1, these enzymes are
overactive, causing tumor cells to grow in an unregulated way
creating so-called plexiform neurofibromas (PN). By blocking these
enzymes, KOSELUGO slows down the growth of tumor cells, and,
therefore, the PN growth.
KOSELUGO is approved in the U.S., European Union (EU), Japan,
China and has been granted Orphan Drug Designation in the U.S., EU,
Japan and other countries for the treatment of pediatric patients
with NF1 PN who have symptomatic, inoperable PN.
KOSELUGO (selumetinib) Indication in the U.S. KOSELUGO is
indicated for the treatment of pediatric patients 2 years of age
and older with neurofibromatosis type 1 (NF1) who have symptomatic,
inoperable plexiform neurofibromas (PN).
IMPORTANT SAFETY INFORMATION WARNINGS AND
PRECAUTIONS Cardiomyopathy. A decrease in left
ventricular ejection fraction (LVEF) ≥10% below baseline occurred
in pediatric patients who received KOSELUGO in SPRINT with some
experiencing decreased LVEF below the institutional lower limit of
normal (LLN), including one patient with Grade 3. All patients with
decreased LVEF were asymptomatic and identified during routine
echocardiography. The safety of KOSELUGO has not been established
in patients with a history of impaired LVEF or a baseline ejection
fraction that is below the institutional LLN. Assess ejection
fraction by echocardiogram prior to initiating treatment, every 3
months during the first year of treatment, every 6 months
thereafter, and as clinically indicated. Withhold, reduce dose, or
permanently discontinue KOSELUGO based on severity of adverse
reaction. In patients who interrupt KOSELUGO for decreased LVEF,
obtain an echocardiogram or a cardiac MRI every 3 to 6 weeks. Upon
resolution of decreased LVEF, obtain an echocardiogram or a cardiac
MRI every 2 to 3 months.
Ocular Toxicity. Blurred vision, photophobia, cataracts,
and ocular hypertension occurred. Retinal pigment epithelial
detachment (RPED) occurred in the pediatric population during
treatment with single agent KOSELUGO and resulted in permanent
discontinuation. Conduct ophthalmic assessments prior to initiating
KOSELUGO, at regular intervals during treatment, and for new or
worsening visual changes. Permanently discontinue KOSELUGO in
patients with retinal vein occlusion (RVO). Withhold KOSELUGO in
patients with RPED, conduct ophthalmic assessments every 3 weeks
until resolution, and resume KOSELUGO at a reduced dose.
Gastrointestinal Toxicity. Diarrhea occurred, including
Grade 3. Diarrhea resulting in permanent discontinuation, dose
interruption or dose reduction occurred. Advise patients to start
an anti-diarrheal agent (eg, loperamide) and to increase fluid
intake immediately after the first episode of diarrhea. Withhold,
reduce dose, or permanently discontinue KOSELUGO based on severity
of adverse reaction.
Skin Toxicity. Rash occurred in 91% of 74 pediatric
patients. The most frequent rashes included dermatitis acneiform
(54%), maculopapular rash (39%), and eczema (28%). Grade 3 rash
occurred, in addition to rash resulting in dose interruption or
dose reduction. Monitor for severe skin rashes. Withhold, reduce
dose, or permanently discontinue KOSELUGO based on severity of
adverse reaction.
Increased Creatinine Phosphokinase (CPK). Increased CPK
occurred, including Grade 3 or 4 resulting in dose reduction.
Increased CPK concurrent with myalgia occurred, including one
patient who permanently discontinued KOSELUGO for myalgia. Obtain
serum CPK prior to initiating KOSELUGO, periodically during
treatment, and as clinically indicated. If increased CPK occurs,
evaluate for rhabdomyolysis or other causes. Withhold, reduce dose,
or permanently discontinue KOSELUGO based on severity of adverse
reaction.
Increased Levels of Vitamin E and Risk of Bleeding.
KOSELUGO capsules contain vitamin E which can inhibit platelet
aggregation and antagonize vitamin K-dependent clotting factors.
Supplemental vitamin E is not recommended if daily vitamin E intake
(including the amount of vitamin E in KOSELUGO and supplement) will
exceed the recommended or safe limits due to increased risk of
bleeding. An increased risk of bleeding may occur in patients who
are coadministered vitamin-K antagonists or anti-platelet
antagonists with KOSELUGO. Monitor for bleeding in these patients
and increase international normalized ratio (INR) in patients
taking a vitamin-K antagonist. Perform anticoagulant assessments
more frequently and adjust the dose of vitamin K antagonists or
anti-platelet agents as appropriate.
Embryo-Fetal Toxicity. Based on findings from animal
studies, KOSELUGO can cause fetal harm when administered during
pregnancy. In animal studies, administration of selumetinib to mice
during organogenesis caused reduced fetal weight, adverse
structural defects, and effects on embryo-fetal survival at
approximate exposures >5 times the human exposure at the
clinical dose of 25 mg/m2 twice daily. Advise patients of
reproductive potential of the potential risk to a fetus and to use
effective contraception during treatment with KOSELUGO and for 1
week after the last dose.
ADVERSE REACTIONS Common adverse reactions ≥40%
include vomiting, rash (all), abdominal pain, diarrhea, nausea, dry
skin, musculoskeletal pain, fatigue, pyrexia, acneiform rash,
stomatitis, headache, paronychia, and pruritus.
DRUG INTERACTIONS Effect of Other Drugs on
KOSELUGO Concomitant use of KOSELUGO with a strong or
moderate CYP3A4 inhibitor or fluconazole increased selumetinib
plasma concentrations, which may increase the risk of adverse
reactions. Avoid coadministration with KOSELUGO. If
coadministration cannot be avoided, reduce KOSELUGO dosage.
Concomitant use of KOSELUGO with a strong or moderate CYP3A4
inducer decreased selumetinib plasma concentrations, which may
reduce KOSELUGO efficacy. Avoid concomitant use with KOSELUGO.
SPECIAL POPULATIONS Pregnancy & Lactation.
Verify the pregnancy status of patients of reproductive potential
prior to initiating KOSELUGO. Due to the potential for adverse
reactions in a breastfed child, advise patients not to breastfeed
during treatment with KOSELUGO and for 1 week after the last
dose.
About the AstraZeneca and Merck strategic collaboration
In July 2017, AstraZeneca and Merck, known as MSD outside of the
United States and Canada, announced a global strategic
collaboration to co-develop and co-commercialize LYNPARZA
(olaparib), a first-in-class PARP inhibitor, and KOSELUGO. Working
together, the companies will develop LYNPARZA and KOSELUGO in
combination with other potential new medicines and as
monotherapies.
Merck’s focus on cancer Every day, we follow the science
as we work to discover innovations that can help patients, no
matter what stage of cancer they have. As a leading oncology
company, we are pursuing research where scientific opportunity and
medical need converge, underpinned by our diverse pipeline of more
than 25 novel mechanisms. With one of the largest clinical
development programs across more than 30 tumor types, we strive to
advance breakthrough science that will shape the future of
oncology. By addressing barriers to clinical trial participation,
screening and treatment, we work with urgency to reduce disparities
and help ensure patients have access to high-quality cancer care.
Our unwavering commitment is what will bring us closer to our goal
of bringing life to more patients with cancer. For more
information, visit https://www.merck.com/research/oncology/.
About Merck At Merck, known as MSD outside of the United
States and Canada, we are unified around our purpose: We use the
power of leading-edge science to save and improve lives around the
world. For more than 130 years, we have brought hope to humanity
through the development of important medicines and vaccines. We
aspire to be the premier research-intensive biopharmaceutical
company in the world – and today, we are at the forefront of
research to deliver innovative health solutions that advance the
prevention and treatment of diseases in people and animals. We
foster a diverse and inclusive global workforce and operate
responsibly every day to enable a safe, sustainable and healthy
future for all people and communities. For more information, visit
www.merck.com and connect with us on X (formerly Twitter),
Facebook, Instagram, YouTube and LinkedIn.
Forward-Looking Statement of Merck & Co., Inc., Rahway,
N.J., USA This news release of Merck & Co., Inc., Rahway,
N.J., USA (the “company”) includes “forward-looking statements”
within the meaning of the safe harbor provisions of the U.S.
Private Securities Litigation Reform Act of 1995. These statements
are based upon the current beliefs and expectations of the
company’s management and are subject to significant risks and
uncertainties. There can be no guarantees with respect to pipeline
candidates that the candidates will receive the necessary
regulatory approvals or that they will prove to be commercially
successful. If underlying assumptions prove inaccurate or risks or
uncertainties materialize, actual results may differ materially
from those set forth in the forward-looking statements.
Risks and uncertainties include but are not limited to, general
industry conditions and competition; general economic factors,
including interest rate and currency exchange rate fluctuations;
the impact of pharmaceutical industry regulation and health care
legislation in the United States and internationally; global trends
toward health care cost containment; technological advances, new
products and patents attained by competitors; challenges inherent
in new product development, including obtaining regulatory
approval; the company’s ability to accurately predict future market
conditions; manufacturing difficulties or delays; financial
instability of international economies and sovereign risk;
dependence on the effectiveness of the company’s patents and other
protections for innovative products; and the exposure to
litigation, including patent litigation, and/or regulatory
actions.
The company undertakes no obligation to publicly update any
forward-looking statement, whether as a result of new information,
future events or otherwise. Additional factors that could cause
results to differ materially from those described in the
forward-looking statements can be found in the company’s Annual
Report on Form 10-K for the year ended December 31, 2023 and the
company’s other filings with the Securities and Exchange Commission
(SEC) available at the SEC’s Internet site (www.sec.gov).
Please see Prescribing Information and Patient Information
(Medication Guide) for KOSELUGO (selumetinib) at
https://alexion.com/Documents/koselugo_uspi.pdf
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