Hughes syndrome is also known as Antiphospholipid syndrome is a disorder that causes an increase in the number of blood clots. This medical condition is one in which the immune system attacks the normal proteins of the body. Antiphospholipid syndrome is an autoimmune, hypercoagulable disease that is caused by the components of the immune system. This can result in the formation of blood clots within the vessels, such as the arteries and veins. This medical condition is associated with some complications, especially in pregnant women. Some of these complications include preeclampsia, premature delivery, miscarriage, and abortion.
Individuals affected by Hughes syndrome have more risk of developing conditions such as
- Deep vein thrombosis: This is a condition in which blood clot develops in the leg.
- Arteria thrombosis: This is a medical condition in which there is a clot in an artery. It can lead to strokes and heart attack.
- Blood clots can also form in the brain, which might lead to conditions such as imbalance, slurred speech, impaired vision and poor memory.
People affected by Antiphospholipid syndrome can be classified into two parts. These are primary and secondary. The primary Antiphospholipid syndrome occurs without other diseases, while secondary Antiphospholipid syndrome occurs in association with other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematous and so on. Antiphospholipid syndrome is capable of causing organ failure, within a short period of time, due to the presence of clots present in the vessels of the body. At this stage, the patients have a high risk of dying from the disease. Physicians generally treat this disease with medications, however, stem cells can also be used for the treatment of this disease. Stem cells are undifferentiated cells in the body that can develop into other types of body cells. The stem cells are extracted from the patient’s body, then he’s made to undergo chemotherapy, which essentially destroys the patient’s immune system
What Are the Causes of Antiphospholipid Syndrome?
Antiphospholipid syndrome is a condition in which there is the formation of clots in the blood, around the body. The body immune antibodies attack the body phospholipids, an important component of the clotting system. Antibodies are a component of the immune system, that fights foreign tissues and microorganisms that enter the body. In this case, the immune system perceives the body phospholipids as foreign materials and sets out to attack them. Antiphospholipid syndrome can be caused by autoimmune diseases or the use of some medications. It can also occur without any underlying trigger.
What Are the Signs and Symptoms of Hughes Syndrome?
Antiphospholipid syndrome is autoimmune and hypercoagulable state disease, so the symptoms of this disease centers around this mechanism. Below are some of the symptoms;
- Deep vein thrombosis: This occurs when there is a formation of a clot in the veins of the leg. Thrombosis basically means the formation of blood clot, while the deep vein indicates that the clot is located in the deep veins of the leg. This could lead to pain when walking, and the swelling of the leg. However, this clots can travel from the leg to other parts of the body, and this is could be life-threatening, especially when the clot travels and blocks the pulmonary or the coronary vessels.
- Stroke: This usually occurs in people affected by the antiphospholipid However, there it’s not associated with cardiovascular diseases.
- Cerebrovascular disease: Antiphospholipid syndrome can also lead to the development of cerebrovascular conditions such as stroke. This is more predominant in young people affected by the disease.
- Skin rash: This is not a common symptom, but it does happen in some cases. Patients present with red rash, that has the appearance of a net. It is also called livedo reticularis.
- Neurological presentations: Patients affected by Antiphospholipid syndrome often presents with some neurological symptoms such as headaches, memory loss, and This is because of the obstruction in some of the vessels of the brain.
- Cardiovascular system: Antiphospholipid syndrome also affects the cardiovascular system. Although this is not very common, the antibodies can damage the valves of the heart, which can subsequently lead to other cardiovascular complications.
- Miscarriages: Antiphospholipid syndrome has a high chance of causing miscarriages and stillbirths. This is due to the high blood pressure during pregnancy caused by the disease.
- Blood loss: Some patients affected by this disease do have a slight reduction in their total platelet count. This kind of patients might not have any noticeable presentations. However, there are some patients that have a huge reduction in their platelet count. This is referred to as thrombocytopenia. Patients in this category might present with bleedings in some parts of the body, such as the gum, the skin etc. They are usually very prone to prolonged bleeding, even after a slight injury
What Are the Risk Factors For Hughes Syndrome?
There is a possibility to have phospholipid antibodies and not present with any symptoms. However, the presence of this risk factors could increase the chances of the formation of blood clot in the vessels;
- Smoking
- Having a high amount of cholesterol in the body, as well as triglycerides increases the risk of having the antiphospholipid syndrome.
- Medications: The consumption of medications such as oral contraceptives and estrogen therapy also increases the chances of having this syndrome.
- Pregnancy: Antiphospholipid syndrome occurs regularly in pregnant women. This might be because of the surge of the body hormones.
- Immobility: Staying in one position for so long, or being on long flights can also predispose people to have this disease.
- Surgery: Surgical procedures could also increase the risk of having this disease.
- Autoimmune disorders: Some autoimmune disorders can trigger the development of the Antiphospholipid syndrome. An example of such disorder is the Systemic lupus erythematosus.
- Race: There is some specific race that has a high chance of developing the Antiphospholipid syndrome.
What Are the Complications of the Hughes Syndrome?
Antiphospholipid syndrome could present with many complications. These complications are due to the formation of clots, and the partial or complete blockage of the flow of blood in the vessels. Some of the complications of this disease are;
- Pregnancy problems: Antiphospholipid syndrome is associated with causing multiple miscarriages in pregnant women. A miscarriage is a pregnancy that is terminated before the 20th week of pregnancy. This syndrome also leads to stillbirths, preterm delivery, and pre-eclampsia.
- Cerebrovascular disorders such as stroke
- Renal failure
- Heart diseases
How Is Hughes Syndrome Currently Treated?
Antiphospholipid syndrome is treated by reducing the abnormal rate of clotting in the blood, and to protect the patients from the complications of the syndrome. Blood thinners are usually administered to reducing clotting. Examples are heparin, warfarin, and aspirin.
Stem Cell Therapy of Hughes Syndrome
Antiphospholipid syndrome occurs due to the attack of normal proteins in the blood, by the autoantibodies. This leads to the hypercoagulable state of the body, leading to the formation of clots in the vessels. Stem cells are undifferentiated cells in the body that can develop into other types of body cells. The stem cells are extracted from the patient’s body, then he’s made to undergo chemotherapy, which essentially destroys the patient’s immune system. The stem cells that were earlier extracted are now implanted to the patient. The new stem cells would normalize the immune system of the patient.
References
Cuadrado, M. (1998). Antiphospholipid, anti-beta 2-glycoprotein-I and anti-oxidized-low- density-lipoprotein antibodies in antiphospholipid syndrome. QJM, 91(9), pp.619-626.
Marmont, A. and Burt, R. (2008). Hematopoietic stem cell transplantation for systemic lupus erythematosus, the antiphospholipid syndrome and bullous skin diseases. Autoimmunity, 41(8), pp.639-647.
Statkute, L. (2005). Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation. Blood, 106(8), pp.2700-2709.
What’s new in the antiphospholipid syndrome?. (2016). Rheumatology.
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