- Altered mental status
- 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)
- Petechiae (pinpoint red or purple spots that appear on the body due to bleeding just under the skin)
- Hemoglobinuria (dark urine)
- Bleeding into the skin or mucus membranes
- Shortness of breath
- Symptoms of kidney failure, such as anuria and swelling over the body
- Tachycardia (fast heart rate)
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
How is thrombotic thrombocytopenic purpura diagnosed?
Doctors will first ask for the signs and symptoms and take your complete medical history.
- 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:
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.
Here are a few of the treatment modalities.
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
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:
- Cyclosporine A
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Wun T. Thrombotic Thrombocytopenic Purpura (TTP). Medscape. https://emedicine.medscape.com/article/206598-overview