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 consequences.
The tiny clots can block blood vessels, which stops your blood from being able to reach your organs. This can compromise the functioning of vital organs such as your heart, brain, and kidneys.
Too many of your blood’s platelets may also combine to form the clots. Your blood might then be unable to form clots when it needs to. For example, if you’re injured, you may be unable to stop bleeding.
The exact prevalence of TTP is unknown, and it varies by geographic location. According to StatPearls, prevalence may range from
If you have TTP, you might notice these skin-related symptoms:
- You might have 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 could look like a rash.
- Your skin may turn yellowish, which is called jaundice.
- Your skin may look pale.
You might also have other symptoms, such as:
In very serious cases, a stroke, major internal bleeding, or a coma can occur.
TTP may be either inherited or acquired.
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 abnormal gene.
The parents don’t usually have symptoms of TTP.
This genetic form of TTP 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.
Enzymes are special proteins that increase the rate of metabolic chemical reactions. Abnormal clotting occurs when the ADAMTS13 enzyme isn’t present.
In other cases, your body mistakenly produces proteins that interfere with the ADAMTS13 enzyme’s job. This is known as acquired TTP.
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 to the development of TTP. These include:
- hormone therapy
- estrogen, as used in birth control or hormone therapy
- cyclosporine (Neoral, Sandimmune), an immunosuppressant drug
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 (RBCs) under a microscope will reveal whether they have damage from TTP. They’ll also look for high levels of bilirubin, a substance that results from the breakdown of RBCs.
Your doctor will also check your blood for:
- antibodies, which are proteins that interfere with the enzyme ADAMTS13
- a lack of ADAMTS13 enzyme activity, which causes TTP
- the enzyme lactate dehydrogenase, which is released from tissue injured by blood clots that are caused by TTP
- high creatinine levels, because TTP can cause kidney problems (which affects the kidneys’ ability to filter creatinine)
- low platelet levels, because the increased clotting causes increased use of platelets
Doctors typically treat TPP by attempting to return your blood clotting ability to normal.
Regardless of whether you have inherited or acquired TTP, you’ll likely need to have treatment every day until your condition improves.
For inherited TTP
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.
For acquired TTP
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 professional will draw your blood, just like when you donate blood.
Afterward, a lab 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 2 to 4 hours.
If the plasma treatment isn’t successful, your doctor may begin treatment with medications to stop your body from destroying the ADAMTS13 enzyme.
In other cases, your spleen might need to be surgically removed. Removal of the spleen can help increase your platelet count.
If you think you might have this condition, don’t delay getting to your doctor or the emergency room.
TTP can be fatal if it’s not found and treated right away, especially when platelet levels are dangerously low. The mortality rate for people who don’t receive treatment is at least 90 percent.
If you get prompt and proper treatment for TTP, it’s likely you’ll recover well from this condition. Between 80 and 90 percent of people survive after receiving proper treatment.
For some people, TTP goes away completely after treatment. For other people, they may have ongoing flare-ups.
If you’re diagnosed with TTP, your doctor will need to check your blood counts regularly.