Blood Disorder: Thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that causes blood clots to form in small blood vessels. This leads to a low platelet count (thrombocytopenia).
Causes
This disease can be caused by a problem with an enzyme (a type of protein) that is involved in blood clotting. This enzyme is called ADAMTS13. The absence of this enzyme causes platelet accumulation. Platelets are particles in the blood that help clotting.
As platelets build up, those available in the blood to help with clotting in other parts of the body decrease. This can cause bleeding under the skin.
In some cases, the disorder is passed from parents to children (hereditary). In these cases, people are born with naturally low levels of this enzyme.
This condition can also be caused by:
- Cancer
- Chemotherapy
- Hematopoietic stem cell transplant
- HIV infection
- Hormone replacement therapy and estrogen
- Medications (including ticlopidine, clopidogrel, quinine, and cyclosporine A)
Symptoms
- Symptoms have been widespread.
- Symptoms may include any of the following:
- Bleeding into the skin or mucous membranes
- Confusion
- Fatigue, weakness
- Fever
- Headache
- Pale or yellowish skin
- Respiratory difficulty
- High heart rate (over 100 beats per minute)
Treatment
- You can receive a treatment called plasma exchange. This treatment removes the abnormal proteins in your plasma and replaces it with normal plasma from a healthy donor. Plasma is the liquid part of blood that contains blood cells and platelets. Plasma exchange also replaces the missing enzyme.
- The procedure is carried out as follows:
- First, your blood will be drawn as if you were donating it.
- As the blood passes through a machine that separates the blood into different parts, the abnormal plasma is removed and your blood cells are saved.
- Your blood cells are combined with normal plasma from a donor, and then returned to your body.
- This treatment is repeated daily until blood tests show improvement.
- People who do not respond to this treatment or whose condition often comes back may need:
- Undergo surgery to remove their spleen
- Receiving medications to suppress the immune system, such as corticosteroids or rituximab
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Expectations (forecast)
- Most people with TTP who undergo plasma exchange recover completely. But some people die from this disease, especially if it is not diagnosed right away. In people who do not recover, this condition may become long-term (chronic).
Complications may include:
- Renal insufficiency
- Low platelet count (thrombocytopenia)
- Low red blood cell count (caused by their premature breakdown)
- Nervous system problems
- Heavy bleeding (hemorrhage)
- stroke
When to contact a medical professional
- Contact your healthcare provider if you have any unexplained bleeding.
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