Substances Antibiotics, Antineoplastic. Immunosuppressive Agents. Muscle Relaxants, Central. Platelet Aggregation Inhibitors. Mitomycin. Cyclosporine. Clopidogrel. Quinine. Ticlopidine. A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes TTP. The ADAMTS13 gene controls the enzyme, which is involved in blood clotting. The enzyme breaks up a large protein called von Willebrand factor that clumps together with platelets to form blood clots. TTP episodes are serious and life-threatening. It is considered a medical emergency and it is estimated that 10-20% of acute patients die from TTP, despite currently available treatments. If a woman has an initial episode of TTP during a pregnancy, inherited ADAMTS13 deficiency should be considered. Therefore acquired TTP is an autoimmune disorder. Autoimmune disorders are more common in women than men, and some autoimmune disorders are more common in specific racial groups. 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 defective gene. The parents don't usually have symptoms of the disease.
Acquired TTP is the more common type that occurs mostly in adults, but it sometimes affects children. The causes are unknown, but it can be triggered by conditions such as pregnancy or infection or diseases such as cancer, HIV, and lupus.
Diagnosis. Laboratory studies for suspected TTP include a CBC, platelet count, blood smears, coagulation studies, BUN creatinine, and serum bilirubin and lactate dehydrogenase. Measuring ADAMTS13 activity level may aid in diagnosis.
In cases when blood platelet count is severely low, it can actually cause internal bleeding in your brain or intestines. Chronic thrombocytopenia may have any of a number of causes. In order for chronic thrombocytopenia to be considered disabling for Social Security Disability purposes, it must be severe.
Thrombocytopenia signs and symptoms may include: Easy or excessive bruising (purpura) Superficial bleeding into the skin that appears as a rash of pinpoint-sized reddish-purple spots (petechiae), usually on the lower legs. Prolonged bleeding from cuts. Bleeding from your gums or nose. Blood in urine or stools.
Thrombotic thrombocytopenic purpura
When you don't have enough platelets in your blood, your body can't form clots. A low platelet count may also be called thrombocytopenia. This condition can range from mild to severe, depending on its underlying cause. For some, the symptoms can include severe bleeding and are possibly fatal if they're not treated.
Purpura occurs when small blood vessels burst, causing blood to pool under the skin. This can create purple spots on the skin that range in size from small dots to large patches. Purpura spots are generally benign, but may indicate a more serious medical condition, such as a blood clotting disorder.
Hereditary thrombotic thrombocytopenic purpura (TTP) may be rare, but it is forever. Hereditary TTP, caused by ADAMTS13 mutations resulting in a severe deficiency of ADAMTS13 activity, is also known as Upshaw-Schulman syndrome.
In the majority of people with ITP, the condition isn't serious or life-threatening. Acute ITP in children often resolves within six months or less without treatment. Chronic ITP can last for many years. People can live for many decades with the disease, even those with severe cases.
Abdominal pain is frequently encountered in patients with thrombotic thrombocytopenic purpura (TTP). TTP should be considered in patients with abdominal pain or pancreatitis in whom thrombocytopenia, microangiopathic hemolytic anemia, neurologic changes, fever, and renal disease are present.
A breakthrough in understanding the pathogenesis of thrombotic thrombocytopenic purpura (TTP) came with the discovery of ADAMTS13 (an abbreviation for “a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13”), a plasma protein that cleaves von Willebrand factor, which interacts with platelets
Lupus is a systemic autoimmune disease that occurs when your body's immune system attacks your own tissues and organs. Inflammation caused by lupus can affect many different body systems — including your joints, skin, kidneys, blood cells, brain, heart and lungs.
ADAMTS13, a protease also known as von Willebrand factor (VWF) cleaving protease, regulates the size of VWF multimers, and is an important mediator of platelet adhesion. ADAMTS13 regulates VWF size by cleaving a specific peptide bond in the A2 domain.