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Symptoms of thrombotic thrombocytopenic purpura (TTP) include bleeding into the skin or mucous membranes, seizures, confusion, and more.
Thrombotic thrombocytopenic purpura (TTP) is a disease of platelets, which are the type of blood cells that help clot blood and prevent abnormal internal bleeding.
Though TTP can affect any organ system, the symptoms result when it affects the skin (purpuric rash), nervous system, and kidneys. The symptoms related to TTP in these cases include:
- Altered mental status
- Seizures
- Hemiplegia
- Paresthesias (numbness or a burning feeling that occurs most often in the hands, arms, legs, or feet)
- Visual disturbance
- Aphasia (language disorder that makes it hard for you to use words)
- Fatigue
- Weakness
- Petechiae (pinpoint red or purple spots that appear on the body due to bleeding just under the skin)
- Fever
- Hemoglobinuria (dark urine)
- Pallor
- Jaundice
- Bleeding into the skin or mucus membranes
- Confusion
- Headache
- Shortness of breath
- Symptoms of kidney failure, such as anuria and swelling over the body
- Tachycardia (fast heart rate)
- Seizures
- Coma
- Stroke
What causes thrombotic thrombocytopenic purpura?
Thrombotic thrombocytopenic purpura (TTP) is caused by a problem in the gene, ADAMTS13, which deals with the clotting of the blood. The absence of this gene hampers the ability of the platelets to aggregate and form a blood thrombus (clot), causing bleeding in several organs of the body, including the skin.
The following causes can predispose you to TTP:
- Heredity (the defective gene passed over to you from your parents)
- Postoperative causes in some surgeries, such as coronary artery bypass grafting for ischemic heart disease, vascular surgery, gastrointestinal surgery, and knee replacements
- Blood and marrow
- Autoimmune causes, such as lupus
- Hormone replacement therapy
- Pills containing estrogens
- Medications
- Chemotherapy
- Ticlopidine
- Clopidogrel
- Quinine
- Cyclosporine A
How is thrombotic thrombocytopenic purpura diagnosed?
Doctors will first ask for the signs and symptoms and take your complete medical history.
The first test that doctors usually order is complete blood count, which will show the following findings if you have thrombotic thrombocytopenic purpura (TTP):
- Normal or high white blood cell count
- Low hemoglobin (Hb) concentration (8 to 9 g/Dl)
- Low platelet count (20,000 to 50,000/Μl)
Based on these results, your doctor may order other tests that include:
- ADAMTS13 activity level
- Bilirubin
- Creatinine level
- Lactate dehydrogenase level
- Urine test
- Haptoglobin
- Coombs test
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How is thrombotic thrombocytopenic purpura treated?
The thrombocytopenia due to platelets can last for days to months. People with mild thrombocytopenia may only need observation. For others, treatment depends on its cause and how severe the symptoms are.
The renal dysfunction that accompanies some cases of thrombotic thrombocytopenic purpura (TTP) may need a team of nephrologists and hematologists for its prognosis.
Here are a few of the treatment modalities.
Plasma therapy
The most common treatment for TTP includes plasma therapy, which involves removing the abnormal liquid part of the blood called plasma and replacing it with normal plasma from a healthy donor. This treatment replaces the enzyme, ADAMTS13.
Plasma therapy is of two types:
- Fresh frozen plasma
- Plasma exchange
Plasma therapy is done in a hospital setting daily until your platelets and other blood cell counts show improvement. You will have to stay in the hospital until you recover.
After recovering from the plasma therapy, you may have flare-ups of TTP. So, you may be asked to get admitted and take the therapy again.
If your condition does not improve with plasma therapy, you may have to try other treatments that include:
- Splenectomy: Surgery to remove their spleen because cells in the organ produce the antibodies that interfere with the ADAMTS13 enzyme activity.
- Medications: Medicines that suppress the immune system and include steroids or rituximab, as well as other medications including:
- Vincristine
- Cyclosporine A
In some cases, treatment of underlying disorders, such as infections or cancer, may improve the symptoms of TTP. The renal dysfunction may require dialysis.
