CSL Behring Announces FDA Approval of Berinert(R), First and Only Therapy Approved for the Treatment of Acute Abdominal and Facial Attacks of Hereditary Angioedema in U.S.

CSL Behring Announces FDA Approval of Berinert(R), First and Only Therapy
Approved for the Treatment of Acute Abdominal and Facial Attacks of Hereditary
Angioedema in U.S.

KING OF PRUSSIA, Pa., Oct. 12 /PRNewswire/ -- CSL Behring announced today that
the U.S. Food and Drug Administration (FDA) has granted marketing approval for
Berinert® C1-Esterase Inhibitor, Human for the treatment of acute abdominal or
facial attacks of hereditary angioedema (HAE), a rare and serious genetic
disorder, in adult and adolescent patients. Berinert is the first and only
therapy approved for this indication in the U.S. The approval is based on the
results of the phase II/III prospective, double-blind placebo-controlled
International Multi-center Prospective Angioedema C1-Inhibitor Trial
(I.M.P.A.C.T.), which studied the efficacy and safety of C1-inhibitor (C1-INH)
concentrate. The safety and efficacy of Berinert for prophylactic therapy have
not been established.

"The FDA approval of Berinert marks an important milestone in CSL Behring's
ongoing commitment to satisfying the unmet needs of patients with rare and
serious disorders, such as hereditary angioedema," said Robert Lefebvre, Vice
President and General Manager of U.S. Commercial Operations at CSL Behring.
"As a leader in developing safe, effective and high-quality therapies, we are
pleased to add to our rapidly growing portfolio a proven treatment that can
make a positive difference in the lives of HAE patients and their families."

HAE is a genetic disorder caused by a deficiency of C1-INH and is inherited in
an autosomal dominant manner. Symptoms of HAE include episodes of edema or
swelling in the face and the abdomen. Patients who have abdominal attacks of
HAE can experience episodes of severe pain, diarrhea, nausea, and vomiting
caused by swelling of the intestinal wall. HAE attacks that involve the face
can cause painful distortion and painful swelling. Diagnosis of HAE requires
a blood test to confirm low or abnormal levels of C1-INH. There are estimates
of 6,000 to 10,000 or more people with HAE in the U.S.

"For individuals with HAE, episodes of swelling can be extremely painful and
frightening, " said Timothy Craig, MD, professor of medicine and pediatrics,
Pennsylvania State University Hershey Medical Center. "With the approval of
Berinert, healthcare professionals can now provide HAE patients in the U.S.
with a safe and effective treatment option that rapidly relieves the symptoms
of acute attacks in the face and abdomen."

"Today's approval provides adult and adolescent HAE patients and their
physicians with a proven, safe, and effective therapy for treating
debilitating, painful, and life-threatening facial and abdominal HAE attacks
once they have begun," said Anthony J. Castaldo, President of the United
States Hereditary Angioedema Association, a nonprofit patient advocacy
organization that represents approximately 6,500 HAE patients in the United
States.

About I.M.P.A.C.T.
I.M.P.A.C.T. was a study of 124 HAE patients with acute, moderate, or severe
abdominal or facial attacks. C1-INH concentrate was administered at two
different doses and compared with placebo. The main study endpoints were time
to onset of symptom relief from HAE attacks, proportion of subjects with
worsening clinical HAE symptoms, and safety.

The I.M.P.A.C.T. study found that C1-inhibitor concentrate (C1-INH) is
effective and safe in rapidly treating acute abdominal and facial skin
swellings in adults and adolescents with HAE. The study found that the median
time to symptom relief was 30 minutes after receiving C1-INH compared with 1.5
hours with a placebo.

About Berinert®
Berinert, a plasma-derived intravenous therapy, treats the fundamental cause
of acute facial and abdominal hereditary angioedema (HAE) symptoms by
providing C1-INH deficient adult and adolescent patients with the missing
human protein. Without C1-INH, patients with HAE suffer from recurrent
episodes of rapid swelling of areas of the skin and underlying tissues
including the face, mouth and abdomen. Berinert( )is a unique HAE therapy
because of its reliable record of proven efficacy and safety in international
clinical use in over 400,000 treatments in Germany, Austria, Switzerland, and
several other countries where it is manufactured and sold by CSL Behring under
the trade name Berinert® P.

Important Safety Information
Berinert is a plasma derived concentrate of C1 Esterase Inhibitor (Human),
indicated for the treatment of acute abdominal or facial attacks of hereditary
angioedema (HAE) in adult and adolescent patients.

The safety and efficacy of Berinert for prophylactic therapy have not been
established. Berinert is contraindicated in individuals who have had an
anaphylactic or severe systemic reaction to C1-INH preparations. Monitor
patients for early signs of allergic or hypersensitivity reactions (including
hives, generalized urticaria, chest tightness, wheezing, hypotension, and
anaphylaxis). If hypersensitivity is suspected, immediately discontinue
administration and initiate appropriate treatment. Epinephrine should be
immediately available for treatment of acute severe hypersensitivity
reactions.

Thrombotic events have occurred in patients receiving off-label high doses of
Berinert. Monitor patients with known risk factors for thrombotic events.

Berinert is derived from human plasma. The risk of transmission of infectious
agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease
(CJD) agent, cannot be completely eliminated.

The most serious adverse reaction reported in subjects in clinical studies who
received Berinert is an increase in the severity of pain associated with HAE.
The most common adverse reactions observed in more than 4 percent of subjects
after Berinert treatment were headache, abdominal pain, nausea, muscle spasms,
pain, diarrhea, and vomiting.

Berinert has not been evaluated in pregnant women or nursing mothers; benefits
of treatment should be weighed against potential risks in pregnant women, and
Berinert should be given to nursing mothers only if clearly needed. Safety and
efficacy of Berinert have not been established in children (ages 0 through 12)
or in the geriatric population. For more information, including full
prescribing information, visit www.berinert.com.

About CSL Behring
CSL Behring is a global leader in the plasma protein biotherapeutics industry.
Passionate about improving the quality of patients' lives, CSL Behring
manufactures and markets a range of safe and effective plasma-derived and
recombinant products and related services. The company's therapies are used in
the treatment of immune deficiency disorders, hemophilia, von Willebrand
disease, other bleeding disorders and inherited emphysema. Other products are
used for the prevention of hemolytic diseases in the newborn, in cardiac
surgery, organ transplantation and in the treatment of burns. The company also
operates one of the world's largest plasma collection networks, CSL Plasma.
CSL Behring is a subsidiary of CSL Limited, a biopharmaceutical company with
headquarters in Melbourne, Australia. For more information, visit
www.cslbehring.com.

Contacts:

Sheila A. Burke, Director
Communications & Public Relations
CSL Behring
+1-610-878-4209 (o)
+1-484-919-2618 (m)
Sheila.Burke@cslbehring.com

Jeff Hoyak
MCS
+1-908-234-9900, ext. 312
jeffh@mcspr.com

SOURCE CSL Behring

Sheila A. Burke, Director, Communications & Public Relations, CSL Behring,
+1-610-878-4209 (o), +1-484-919-2618 (m), Sheila.Burke@cslbehring.com; or Jeff
Hoyak, MCS, +1-908-234-9900, ext. 312, jeffh@mcspr.com