acquired Thrombotic thrombocytopenic purpura

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acquired Thrombotic thrombocytopenic purpura

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Acquired thrombotic thrombocytopenic purpura is a blood disorder characterized by thrombocytopenia (low number of platelets), bleeding under the skin (purpura), decreased red blood cell count, and hemolytic anemia. The disorder causes widespread formation of blood clots (thrombi) in small blood vessels throughout the body. These clots can give rise to serious medical problems if they obliterate blood vessels and decrease or stop the flow of blood to vital organs such as the brain, kidneys, and heart. Acquired thrombotic thrombocytopenic purpura usually begins in adulthood, but can affect children as well. An episode usually occurs suddenly and lasts for few days or weeks, but it may continue for many months altogether. In about 60% of people who have the disease, relapses can occur.


Acquired thrombotic thrombocytopenic purpura is caused when a person's body mistakingly makes auto-antibodies that disrupt or block the activity of the ADAMTS13 enzyme. The ADAMTS13 enzyme helps control the activity of certain blood clotting factors.

Signs and symptoms:

The signs and symptoms of acquired thrombotic thrombocytopenic purpura include purplish bruises on the skin or mucous membranes, pinpoint-sized red or purple dots on the skin, paleness, jaundice, fatigue (feeling very tired and weak), fever, shortness of breath, fast heart rate, nausea, vomiting, headache, speech changes, stroke, confusion, coma, or seizure.

Acquired thrombotic thrombocytopenic purpura usually presents as severe low red blood cell count due to destruction of red blood cells and low platelet count in a previously healthy individual. This medical disorder adversely affects the central nervous system and gastrointestinal system in over 1/3 of cases. Kidneys may be damaged, but severe kidney damage is no longer common. Heart may be affected, but the lungs are rarely affected.


Complications may include neurological problems, such as headaches, confusion, personality changes, and slurred speech. Other complications include fever, renal insufficiency, abdominal pain, and heart problems. Hemolytic anemia can be caused, leading to paleness, shortness of breath, yellowing of the eyes and skin (jaundice), fatigue, and a rapid heart rate.


Acquired thrombotic thrombocytopenic purpura is diagnosed by careful medical history, a thorough physical exam, and blood tests. Medical history may reveal having had certain diseases or conditions, which can be triggers for the onset of acquired thrombotic thrombocytopenic purpura, such as cancer, HIV, lupus, or recent infections. Certain medications (quinine), chemotherapy and procedures (bone marrow transplants) may also trigger the onset of this disorder. Physical exam is focused at finding the clinical signs of the disease. Blood tests are used to measure red blood cell count, platelet and white blood cell counts, rule out other causes of anemia, and evaluate kidney function. Special blood test assays can be used to check the activity of the ADAMTS13 enzyme and to explore the presence of the ADAMTS13 antibody inhibitor.


The mainstay of treatment for people with acquired thrombotic thrombocytopenic purpura is daily plasma exchange (plasmaphoresis). This procedure removes antibodies (proteins) from the blood that damage the ADAMTS13 enzyme. Corticosteroids are also given in addition to plasmapheresis. Rituximab is used in cases that are refractory, not responding to treatment well, or are very severe. Plasmapheresis is recommended to be continued daily until the platelet count has normalized. Once the clinical condition of the person stabilizes, corticosteroids will be decreased over a 2-3 week period. Unfortunately, in about 10% of cases, acquired thrombocytopenia does not show encouraging clinical response to these treatments. Other modalities include certain chemotherapy drugs or increase in the dose of corticosteroids.

Other names of the condition:

Thrombotic thrombocytopenic purpura, acquired

Purpura, thrombotic thrombocytopenic


Moschowitz syndrome

Idiopathic thrombotic thrombocytopenic purpura