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Author Topic: Antiphospholipid Antibody Syndromes (APS)  (Read 8568 times)
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« on: February 16, 2006, 07:03:05 pm »

What is Hughes syndrome?

Hughes syndrome, also known as antiphospholipid syndrome or 'sticky blood' is a disorder characterized by blood clotting, both in the arteries and veins.

What causes Hughes Syndrome?
Hughes syndrome is caused by the presence of antibodies in the blood, called antiphospholipid antibodies. These antibodies make the immune system work too hard, increasing blood clotting. Blood clotting can affect anyone of any age and can happen suddenly e.g. leg thrombosis or over a number of years. For more information on deep vein thrombosis (DVT) click here.

What are the symptoms of Hughes Syndrome?
In some people the only symptom of Hughes syndrome is recurrent miscarriage (often late in pregnancy). This is due to small clots affecting the placenta, preventing vital nutrients getting to the growing baby. However, other symptoms could include any of the following:
Headaches (migraine type).
Epilepsy.
Memory loss.
Blotchiness of the skin, which causes bluish discolouration of certain areas of the body, such as the knees and wrists.
Hughes syndrome is also an important cause of thrombotic disease, including thrombosis of the leg or arm or of any internal organ. As a result there is a possibility it can cause strokes and heart attacks.

Am I at risk of Hughes Syndrome?
There is a strong link between people with lupus and those with Hughes syndrome, 1 in 5 women with lupus also have Hughes syndrome. Please click here to read more about lupus and the symptoms associated with lupus.
However, there are many more people who have Hughes syndrome who never go on to develop lupus. Hughes syndrome and lupus are both more common in women.

How is Hughes Syndrome diagnosed?
Hughes syndrome is diagnosed by blood tests, one which looks for antiphospholipid antibodies and the other is called lupus anticoagulant, which will help identify if lupus is present. Your doctor may only carry out one test. The diagnosis will depend greatly on your symptoms and past medical history.

How is Hughes Syndrome treated?
As the main symptoms of Hughes syndrome is the increased tendency of blood clots, the treatment is aimed at thinning the blood to reduce the risk. Treatment to thin the blood may be with drugs, such as aspirin. Aspirin makes the blood less sticky, as well as cutting down the chances of thrombosis and strokes.

There are also stronger blood thinners available, such as heparin or warfarin depending on how severe the condition is. If you are trying to become pregnant warfarin should not be taken as it is potentially toxic to the developing fetus.

If you are taking warfarin regular blood tests are required to ensure the drug is thinning the blood. Heparin needs to be given daily but unfortunately is only available as an injection and cannot be given long term.
If you have been diagnosed with Hughes syndrome you should limit the risk of thrombosis from other causes, for example quit smoking, keep to a healthy weight and get regular exercise.

If you believe you are at risk of Hughes Syndrome then speak to your doctor for advice and help. For further information on Hughes syndrome you can also try the following website : www.hughes-syndrome.org.
« Last Edit: May 17, 2006, 06:11:18 pm by Kathy » Logged


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« Reply #1 on: October 08, 2006, 10:15:45 am »

Antiphospholipid Antibody Syndromes (APS)

Antiphospholipid Antibody Syndrome (APS) is an autoimmune disease. In this disorder antibodies, which are found in the blood and normally fight off infections, are instead turning against the body's own normal clotting mechanisms. These antibodies make people more prone to certain problems, such as clots in the deep veins in the arms and legs, known as Deep Vein Thrombosis (DVT) or in the lung, known as Pulmonary Embolism (PE). People with APS can also develop clots in their arteries, known as heart attacks or strokes. In certain women, these antibodies can cause recurrent pregnancy loss.

Physicians use a combination of clinical symptoms, such as blood clots, and specific laboratory tests to diagnose the presence of APS. Laboratory studies used in the diagnosis of APS include tests for lupus anticoagulants and anticardiolipin antibodies. Although many patients with APS have lupus, most patients don't (referred to as primary APS).

The treatment of choice for patients with APS who have had a blood clot is anticoagulant therapy ('blood thinner'). For women with APS and recurrent miscarriages who have not had a prior blood clot, the use of anticoagulant therapy during the pregnancy may increase the likelihood of a successful outcome. Some individuals may have elevated antiphospholipid antibodies but have no clinical manifestations of the syndrome. These individuals do not need anticoagulant therapy, but studies are ongoing to evaluate whether an aspirin a day might be beneficial for these individuals.

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« Reply #2 on: October 08, 2006, 10:18:30 am »

Catastrophic Antiphospholipid Syndrome

The catastrophic antiphospholipid syndrome is a very rare complication encountered in a subset of patients with antiphospholipid antibody syndrome. This rare syndrome is characterized by the development of multiple blood clots that block small blood vessels in several organs in the body. The organs most commonly affected by these small blood clots include the heart, lungs, nervous system, and kidneys. In many ways, this syndrome is similar to another rare disease, thrombotic thrombocytopenic purpura.

Many patients who develop this rare complication have lupus, and infections have been reported to potentially increase a patient's risk to develop the syndrome. Even with the best treatment, as many as half the patients who develop this syndrome do not survive.

Treatment includes anticoagulation (blood thinners), steroids, and a procedure called 'plasma exchange'. Plasma exchange refers to a process whereby a patient's plasma (the liquid part of the blood) is removed and replaced with plasma from blood donors. Patients who survive this life-threatening complication are generally maintained on long-term anticoagulant therapy.

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« Reply #3 on: October 08, 2006, 10:20:09 am »

Thrombotic Thrombocytopenic Purpura (TTP)
Thrombotic thrombocytopenic purpura ("TTP") is a rare blood condition characterized by a low platelet count and the widespread formation of small blood clots throughout the circulation. Platelets are the smallest of the cells in the blood, and they function as tiny corks that form a plug to stop bleeding. In general, a very low platelet count, referred to as 'thrombocytopenia', is associated with bleeding problems, and these patients may present with large bruises, or 'purpura'. These patients also develop clotting complications, however, involving the brain, kidneys, and virtually any organ in the body. These small clots are referred to as 'thrombi'.

TTP may occur in one to three people per million every year and appears to occur more frequently in women than in men. There is a very rare inherited form, but most cases of TTP appear to develop spontaneously. In some cases, the development of TTP may be associated with certain medicines or other clinical conditions, such as during pregnancy or with lupus or cancer. Symptoms of TTP can be subtle, and may include fever, fatigue, headache or bruising. Some patients may present with kidney failure or stroke-like symptoms, and the disease can be fatal if it is not quickly diagnosed.

TTP is diagnosed by the clinical presentation and certain associated laboratory findings. In particular, what distinguishes this disorder from the many other disorders that cause a low platelet count is that the patient's red blood cells will look torn or fragmented under the microscope. These damaged red blood cells are referred to as "schistocytes".

Treatment of TTP requires specialist care at a Medical Center. The primary treatment for TTP is a process called "plasma exchange", during which a patient's plasma (the liquid part of the blood) is removed and replaced with plasma from blood donors. This treatment is very effective and life-saving in the majority of patients. Other treatments that have been used include steroids, aspirin, and certain chemotherapy agents. In a subset of patients, the TTP may recur after stopping treatment. These patients may require treatment to be restarted, or a different type of therapy may become necessary.

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« Last Edit: October 09, 2006, 08:26:08 am by ♥ Supreme Queen Goddess ♥ » Logged


I look normal, as I have an "Invisible Illness". You can not catch it, you can not see it. It's called Lupus.My body is attacking itself on the inside.
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