What is thrombotic thrombocytopenic purpura (TTP)?
Thrombotic thrombocytopenic purpura
(TTP) is a rare disorder that affects your blood’s tendency to clot. In this
disease, tiny clots form throughout your body. These tiny clots have major
The tiny clots can block blood
vessels. This stops your blood from being able to reach your organs. This can
compromise the functioning of your vital organs, such as your heart, brain, and
The tiny clots can also use up too
many of your blood’s platelets. Your blood might then be unable to form clots
when it needs to form them. For example, if you’re injured, you may be unable
to stop bleeding.
What are the symptoms of TTP?
If you have TTP, you might have these symptoms on your skin:
- You might notice bruises that are purplish in
color and have no obvious cause. These marks, called purpura, are part of what
gives this condition its name.
- You might also have tiny red or purple spots
that might look like a rash.
- Your skin may turn yellowish, which is called
- Your skin may look pale.
You might also have other symptoms, such as:
- a fever
- a headache
In very serious cases, a stroke, major internal bleeding, or
a coma can occur.
What causes TTP?
There’s an inherited form of TTP that
transmits as an autosomal recessive trait. This means that both parents of an
affected individual must carry a copy of the defective gene. The parents don’t
usually have symptoms of the disease. This genetic form results from a mutation
in the ADAMTS13 gene. This gene plays a role in the production of an enzyme
that causes your blood to clot normally. Abnormal clotting occurs when this
enzyme isn’t present. Enzymes are special proteins that increase the rate of
metabolic chemical reactions.
In other cases, your body mistakenly
produces proteins that interfere with the enzyme’s job. This is known as
You can get acquired TTP in a variety
of ways. You can develop it if you have HIV, for example. You can also develop
it after certain medical procedures, such as a blood and marrow stem
cell transplant and surgery.
In some cases, TTP can develop during
pregnancy or if you have cancer or an infection.
Some medications can lead you to
develop TTP. These include:
- hormone therapy
- cyclosporine A
How is TTP diagnosed?
Diagnosing TTP usually requires multiple tests. Your doctor
may begin by performing a physical exam. This involves looking for any of the
physical symptoms of the disease.
Your doctor will also test your blood. An examination of
your red blood cells under a microscope will reveal whether they have damage
from TTP. Your doctor will also check your blood for low platelet levels
because the increased clotting causes increased use of platelets. They’ll also
check for high levels of bilirubin, which is a substance that results from the
breakdown of red blood cells.
Your doctor will also check your blood for:
- antibodies, which are proteins that interfere
with the enzyme ADAMTS13
- creatinine levels, which increase if there’s
kidney involvement due to the breakdown of muscle
- a lack of ADAMTS13 enzyme activity, which causes
- lactate dehydrogenase, which is released from tissue
injured by blot clots that are caused by TTP
How is TTP treated?
Doctors typically treat TPP by attempting to return your
blood clotting ability to normal.
The usual treatment for inherited TTP is to administer
plasma intravenously, or through an IV. Plasma is the liquid portion of blood
that contains essential clotting factors. You can receive it in the form of
fresh frozen plasma.
An alternative treatment that’s common for acquired TTP is
plasma exchange. This means that plasma from a healthy donor replaces your plasma.
During this procedure, a healthcare provider will draw your blood, just like
when you donate blood. In a lab, a technician will separate the plasma from
your blood using a special machine called a cell separator. They’ll replace
your plasma with donated plasma. You’ll then receive this new solution through
another IV. The donated plasma contains water, proteins, and essential clotting
factors. This procedure takes about two hours.
Regardless of whether you have inherited or acquired TTP,
you’ll likely need to have treatment every day until your condition improves.
If the plasma treatment isn’t successful, your doctor may
begin to treat you with medications to stop your body from destroying the
In other cases, your spleen might need to be surgically
What is the long-term outlook?
TTP can be fatal if it’s not found and
treated right away, especially when platelet levels are dangerously low. If you
think you might have this condition, don’t delay in getting to your doctor or
the emergency room.
If you get prompt and proper treatment
for TTP, it’s likely you’ll recover well from this condition.
Some people’s TTP goes away completely
after treatment. Other people may have ongoing flare-ups. Your doctor will need
to check your blood counts regularly.