Healthline
VOLUME 18
Coram’s Continuing Education Program
Factor XIII Deficiency (Fibrin Stabilizing Factor Deficiency)
Factor XIII (FXIII) is a protein made
by the body that stabilizes the
formation of a blood clot. When
FXIII is deficient, a clot will still
develop, but it will remain unstable
and the formed clot will eventually
break down and cause recurring
bleeds. FXIII is vital in the process
of coagulation, as evidenced by
symptoms that occur in patients
who are homozygously deficient in
FXIII or have antibodies that disrupt
the function of FXIII. FXIII is clearly
involved in the clot preservation
side of the delicate balance
between clot formation and stability
and clot degradation.
FXIII deficiency, also called fibrin
stabilizing factor deficiency, is the
rarest of all factor deficiencies
with a national estimate of one in
five million births. FXIII deficiency
affects both males and females
equally, as well as all racial and
ethnic groups. FXIII deficiency
can be acquired or inherited.
When inherited, it is an autosomal
recessive disease. Bleeding caused
by a deficiency of FXIII is typically
associated with trauma.
Inherited FXIII
Deficiency
A carrier of FXIII deficiency is
described as an individual who is
not affected by the disease, but
who carries the defective gene. If
the parents of an individual with
inherited FXIII deficiency do not
have the condition themselves,
they would both be considered
carriers of the defective gene.
Inherited FXIII deficiency can be
passed on in the following ways:
• Two parent carriers would
have a:
• 1 in 4 chance of having a
child with severe factor XIII
deficiency
• 1 in 2 chance of having
a child who is a carrier
• 1 in 4 chance of having
a child who is normal
• One parent with factor XIII
deficiency and one parent
who is a non-carrier (normal)
would have:
• Children who are carriers
• One parent with factor XIII
deficiency and one parent who
is a carrier would have a:
• 1 in 2 chance of having a
child who is a carrier
• 1 in 2 chance of having
a child with factor XIII
deficiency
FXIII Deficiency and
Cause of Bleeding
Clotting is a very complex process
that makes it possible to stop an
injured blood vessel from bleeding.
When a blood vessel is injured, the
coagulation process starts to form
a plug. The platelets are first to
arrive at the injury site; they clump
together and stick to the wall
of the vessel. The platelets then
give off chemical signals for help
to other platelets, as well as to
clotting factors. Clotting factors
are tiny plasma proteins that link
together to form fibrin, strands
of plasma proteins that weave
a mesh around the platelets to
prevent them from returning to
the bloodstream. Should the fibrin
mesh break, bleeding will reoccur;
this can occur hours or days postinjury. FXIII helps stabilize the fibrin
mesh by making the individual
fibers solid, which helps prevent
them from breaking away.
The FXIII protein consists of two
parts: one part is produced in the
bone marrow, and the other in the
liver. A liver transplant can serve
as a cure for individuals diagnosed
with FVIII or IX deficiency, because
those factors are produced in the
liver. However, because FXIII is
produced in both the liver and bone
marrow, no cure is available.
Diagnosis
When abnormal bleeding is
identified in a patient, the first test
used is a clotting test. However,
this standard test cannot be
used to diagnose FXIII deficiency,
as a normal result will show in
the presence of this condition.
Therefore, the following tests are
used to diagnose FXIII:
• Stability testing: This is the
most commonly used screening
method. A blood sample is
taken and allowed to clot. Once
the clot forms, it is suspended
in a solution. When FXIII is
present, the clot remains stable
after 24 hours. If FXIII is not
present, the clot dissolves
within minutes. FXIII levels of
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1% to 3% can be enough to
stabilize the clot. For patients
with milder FXIII deficiencies
or those who have had recent
transfusions, the results may still
be normal, despite the presence
of the FXIII deficiency.
• Assay tests: These two tests
assess the activity of FXIII by
measuring the amount of certain
chemicals that are produced
when FXIII is working correctly.
• Enzyme-linked
immunosorbent assay
(ELISA): This test is used to
determine the levels of FXIII in
the blood. If FXIII is present, the
solution will change color.
Symptoms
In 80% of newborn FXIII deficiency
cases, persistent bleeding from the
umbilical stump is found in the first
few days after birth. Bleeding may
also be experienced in soft tissue
and will appear as a bruise, which
is another common symptom of
FXIII deficiency.
Less common bleeds that occur in
about 30% of patients with severe
FXIII deficiency include:
• Central nervous system
bleeding (brain or spinal cord),
which can occur with or without
injury and is considered life
threatening
• Mouth bleeds
• Intramuscular bleeds
(accumulation of blood in a
muscle), which can occur
after strenuous exercise even
without injury
• Bleeding from a laceration (cut)
• Bleeding around the joints,
which is called periarticular
bleeding (bleeding into joints,
which is called hemarthrosis,
is rare)
• Problems during pregnancy
(including recurrent
miscarriages)
• Poor wound healing
2
• Abnormal bleeding during or
after surgery or injury
Treatment
To control and prevent bleeding
due to FXIII deficiency, a slight
increase in the amount of FXIII is
required. Also, preventive therapy is
recommended, due to the high risk
of bleeding into the brain. Infusion
therapy is provided to patients with
FXIII deficiency regardless of signs
of bleeding.
In the past, the recommended
treatment was to provide plasma
and cryoprecipitate to patients
on a monthly basis. Due to the
risk of viral transmission and
severe allergic reaction, both of
these treatments are no longer
recommended. Plasma is also no
longer recommended because of
the variability in the amount of FXIII
available in each bag of plasma.
The current recommended
treatment is FXIII concentrate.
This is a frozen dried powder that
is mixed with sterile water and
then infused. The concentrate is
developed from donated pooled
plasma and is then progressed
through a viral inactivation process,
where it is tested to ensure that it is
free of viruses.
The half-life of FXIII is 8 to 10 days,
which means that half of the FXIII
administered disappears by 8 to
10 days after infusion. After about
28 to 30 days, the factor XIII level
should be back to the original
diagnosed level. This is why
monthly treatments are necessary
to stabilize any abnormal bleeding.
Half-life testing is required prior to
establishing a treatment plan.
Similar to treating for FVIII and FIX
deficiencies, the entire vial should
be infused, as a slightly higher
dose than targeted is harmless.
FXIII patients benefit from selfinfusion education, which helps
foster independence and helps
eliminate the inconvenience
of visiting a healthcare center
monthly for prophylactic treatment.
However, should an injury occur, it
is vital that the patient notifies his
or her physician, and should be
seen at a hospital for evaluation
and possible treatment.
The two concentrates available
and approved by the FDA are
Corifact®, a plasma derived
product by CSL Behring and
Tretten®, a recombinant product by
NovoNordisk.
Bleeding Episode Facts
for Factor XIII
FXIII deficiency bleeds are different
than those caused by hemophilia.
• Bleeding from the umbilical cord
and intracranial hemorrhages
are the most common bleeding
episodes experienced by
patients with FXIII deficiency.
Corifact® Factor XIII Concentrate Adult Dosing and Uses
Dosing Form and Strengths
Powder for reconstitution, injection: 1,000–1,600 units/single-use vial
Congenital Factor XIII Deficiency
Indicated for routine prophylaxis of congenital factor XIII deficiency
Initial dose: 40 units/kg IV; infuse at rate not to exceed 4 mL/min
Subsequent doses are administered q28d and are based on maintaining trough FXIII activity level at
~5–20% (use Berichrom® activity assay)
Dosing adjustments:
yy One trough level <5%: Increase by 5 units/kg
yy Trough level 5–20%: No change
yy Two trough levels >20%: Decrease by 5 units/kg
yy One trough level >25%: Decrease by 5 units/kg
Coram’s Continuing Education Program
• Joint and muscle bleeds
are less frequent with FXIII
deficiency than with FVIII or FIX
deficiencies.
• Prophylactic therapy is more
effective with FXIII deficiency
due to the half-life of FXIII,
which is 8 to 10 days. Based on
the results of previous studies
on FXIII, there is almost no
bleeding when FXIII concentrate
is given in adequate doses at
suitable intervals.
• In FXIII deficiency, as with FVIII
and IX deficiency, any bleeds
to the head, neck, chest or
abdomen are life-threatening
and require immediate medical
attention. The following are
signs and symptoms for lifethreatening bleeds:
• Head—headache, nausea
and vomiting, vision
changes, sleepiness
• Neck—pain in neck or
throat, swelling, difficulty
swallowing and breathing
• Chest—pain in chest,
coughing or spitting up
blood, difficulty breathing
• Abdomen—abdominal or
low back pain, nausea or
vomiting, blood in urine or
stool
• In soft tissue bleeds, look for an
increase at the injury site of the
following:
• Redness
• Discoloration
• Swelling and pain
• Signs of joint bleeds include:
• Pain
• Swelling
• Decreased range of motion
• Limited use of affected joint
and/or extremity
The R.I.C.E method should be used
to reduce the pain and discomfort
of joint or muscle bleeds:
• Rest: Rest the affected
extremities with the use of
crutches or a sling.
• Ice: Apply ice to the
injured site, usually for 15
minutes every 2 hours, to
help reduce swelling at the
affected area.
• Compression: Apply
compression to the affected
limb by using an elastic
bandage. This will also
help reduce swelling.
Note: After applying the
bandage, check the fingers
or toes for numbness,
changes in temperature, or
tingling. These symptoms
could be caused if the
bandage is applied too
tightly. If these symptoms
occur, loosen or remove
the bandage to see if the
symptoms resolve.
• Elevation: Raising the
affected extremity above
the heart can help decrease
swelling and improve
circulation.
Factor XIII — An Inherited Bleeding
Disorder. (2007) Canadian Hemophilia
Society.
4. Medscape (2012) Factor XIII Concentrate,
Human (Rx). Retrieved in 2012. Accessed
from http://reference.medscape.com/
drug/corifact-factor-xiii-concentratehuman-999624.
5. Schwartz R and Besa E. Factor XIII.
(July 11, 2012). Retrieved in 2012.
Accessed from http://emedicine.
medscape.com/article/
209179-overview.
Conclusion
FXIII deficiency is a rare disorder
that affects both females and
males equally. As with hemophilia,
patients with FXIII deficiency have
the risk of life-threatening bleeding,
which requires immediate medical
attention and factor infusion. With
the use of prophylactic therapy
for FXIII deficiency, patients
can lead a normal lifestyle and
participate in sports if approved
by their physician. As with patients
who have any bleeding disorder,
individuals with FXIII deficiency
should be monitored at a facility
that is knowledgeable and
specializes in the treatment of
bleeding disorders.
References:
1. NHF (2006) Factor XIII Deficiency.
Retrieved in 2012. Accessed from
www.hemophilia.org.
2. Factor XIII (2012). What is Factor XIII?
Retrieved in 2012. Accessed from
http://factorxiii.org/overview.htm.
3. Brackmann H and Ivaskevicius V.
Volume 18 3
Self-Assessment Quiz: Factor XIII Deficiency (Fibrin Stabilizing Factor Deficiency)
LEARNING GOAL
LEARNING OBJECTIVES
To understand factor XIII or fibrin
stabilizing factor deficiency, its
contributing genetic factors,
symptoms of bleeds, and treatment
methods with prophylactic use of
factor XIII concentrates.
Upon completion of this continuing education program, the reader will be
able to:
1. Understand and recognize early signs of factor XIII deficiency.
2. Identify signs and symptoms of bleeding episodes.
3. Determine treatment options for factor XIII deficiency patients.
SELF-ASSESSMENT QUESTIONS
In the Quiz Answers section on the next page, circle the correct answer for each question. To obtain two (2.0)
contact hours toward CE credit, the passing score is 100%. Return your Self-Assesment Quiz to Coram via email,
fax or mail. See the next page for details on how to return to your quiz. Please allow approximately seven days to
process your test and receive your certificate upon achieving a passing score.
1. Factor XIII deficiency:
a.Is rare
b.Is equal in females and males
c.Affects all racial and ethnic groups
d.All of the above
2. To inherit factor XIII deficiency, both parents must
be carriers.
a.True
b.False
3. The factor XIII protein is found in the:
a.Liver
b.Spleen
c.Bone marrow
d.A and C
4. The most common symptom of factor XIII deficiency
is:
a.Joint bleed
b.Muscle bleed
c.Persistent bleeding from the umbilical stump
after birth
d.Central nervous system bleeds
6. A person with less than 2% of the normal amount
of factor XIII is considered to have factor XIII
deficiency.
a.False
b.True
7. After surgery, bleeding due to factor XIII deficiency
will occur hours to days after the procedure instead
of during surgery.
a.False
b.True
8. The usual prophylactic therapy for a patient with
factor XIII deficiency is a weekly infusion.
a.False
b.True
9. Liver transplant is a cure for factor XIII deficiency.
a.False
b.True
10.The stability test is the most commonly used
screening method.
a.False
b.True
5. The half-life of factor XIII is:
a.10–12 hours
b.5–8 days
c.24–28 hours
d.8–10 days
4
Coram’s Continuing Education Program
Healthline
VOLUME 18
Coram’s Continuing Education Program
Factor XIII Deficiency (Fibrin Stabilizing Factor Deficiency)
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