Bay Biosciences provides high-quality, fresh, frozen sera (serum), plasma, peripheral blood mononuclear cells (PBMC) bio-fluids from patients diagnosed with thrombotic microangiopathies (TMA) syndromes.
Sera (serum), plasma and PBMC biofluid specimens are processed from thrombotic microangiopathies (TMA) syndromes patient’s peripheral whole-blood using customized collection and processing protocols.
Thrombotic Microangiopathies (TMA) Overview
Thrombotic microangiopathies (TMA) are clinical syndromes defined by the presence of hemolytic anemia (destruction of red blood cells), low platelets, and organ damage due to the formation of microscopic blood clots in capillaries and small arteries.
The kidneys are commonly affected, although virtually any organ may be involved. Smoldering TMA will sometimes result in kidney damage without significant anemia or low platelets.
Under the microscope, the blood demonstrates injured red blood cells known as schistocytes or fragments. Kidney disease can be severe, with over fifty percent of the TMA patients requiring dialysis with a cause of TMA known as atypical hemolytic uremic syndrome.
Earlier, TMA’s were often referred to as TTP or HUS, or thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS). It is now recognized that a large number of different diseases and syndromes can result in TMA.
How Thrombotic Microangiopathies (TMA) Affects Kidneys
In the normal kidney, as in the rest of the body, there are small blood vessels called capillaries. They are lined with a slippery coating of cells known as endothelial cells. When the endothelial cells of capillaries become damaged, blood flow through the kidney slows.
The liquid part of the blood (plasma) helps waste flow to the kidney to be removed from your body through urine.
There are also solid particles in the blood, including red blood cells and platelets. Red blood cells carry oxygen from the lungs to the rest of your body, including the cells of the kidney. Platelets have the job of plugging up any damaged part of the blood vessel to keep it from leaking.
Defects in the blood vessel wall lining can produce rough patches that are like potholes on a road, they can slow traffic and cause a lot of damage. Red blood cells can become deformed and then burst. Platelets can activate and cause clots to form. The wreckage from all these events can close off entire blood vessels. In the end, parts of the kidney can die from lack of blood flow, and the body can run low on red blood cells and platelets.
Thrombotic Thrombocytopenic Purpura (TTP)
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder that affects the blood’s tendency to clot. In this disease, tiny clots form throughout the patients body, these tiny clots have major consequences.
Exact prevalence of TTP is unknown, and it varies by geographic location. According to the NCBI , the prevalence of TTP in patients may range from1 to 13 cases per 1 million patients.
The tiny clots can block blood vessels, which stops the blood from being able to reach different organs. This can compromise the functioning of vital organs such as the heart, brain, and kidneys.
Excess of the blood’s platelets may also combine to form the clots. In patients wit TTP the blood might then be unable to form clots when it needs to. For example, if the TTP patient gets injured, they would not be able to stop bleeding.
Causes of Thrombotic Microangiopathies (TMA)
TMA are associated with a large number of diseases. A common cause is thrombotic thrombocytopenic purpura (TTP) which is due to low activity of a protein known as ADAMTS13.
Some people are born with a mutation in the gene for ADAMTS13, although most affected patients have an acquired auto-antibody (a form of autoimmune disease) that blocks the activity of ADAMTS13.
Another major cause for TMA is atypical hemolytic uremic syndrome (aHUS), a disorder caused by dysregulation of a part of the immune system known as complement.
Approximately half of the aHUS patients are found to have either a genetic mutation in the complement system or an auto-antibody that interferes with the regulation of complement.
Other notable causes for TMA include infection (e.g., bloody diarrhea associated with E. coli infection), medications (such as quinine, bevacizumab), connective tissue diseases, e.g., Systemic Lupus Erythematosus (SLE), antiphospholipid antibody syndrome, scleroderma, cancer, vasculitis, pregnancy, malignant hypertension, organ transplant, and metabolic disorders.
Inherited Thrombotic Thrombocytopenic Purpura (TTP)
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 abnormal gene. The parents usually don’t have symptoms of TTP.
This genetic form of TTP results from a mutation in the ADAMTS13 gene. This gene plays a role in the production of an enzyme that causes the blood to clot normally.
Enzymes are special proteins that increase the rate of metabolic chemical reactions. Abnormal clotting occurs when the ADAMTS13 enzyme is not present.
Acquired Thrombotic Thrombocytopenic Purpura (TTP)
In other cases, the body mistakenly produces proteins that interfere with the ADAMTS13 enzyme’s job. This is known as acquired TTP.
Individuals can get acquired TTP in a variety of ways. Patients can develop TTP if they have HIV, for example. You can also develop it after certain medical procedures, such as a blood and marrow stem cell transplant and surgery.
In some cases, TTP can develop during pregnancy or if you have cancer or an infection.
Following are some of the treatments that can lead to the development of TTP:
- Chemotherapy
- Hormone Therapy
- Estrogen , as used in birth control or hormone therapy
- Cyclosporine (Neoral, Sandimmune), which is an immunosuppressant drug.
Signs and Symptoms of Thrombotic Thrombocytopenic Purpura (TTP)
Following are some of the signs and symptoms of Thrombotic Thrombocytopenic Purpura (TTP) usually these are skin-related conditions:
- Bruises that are purplish in color and have no obvious cause. These marks, called purpura, are part of what gives this condition its name.
- Red or purple spots that could look like a rash.
- Skin may turn yellowish (Jaundice)
- The skin may look pale
Some of the Thrombotic Thrombocytopenic Purpura (TTP) patients may also have other following symptoms:
In very serious cases, a stroke, internal bleeding or a coma can happen.
Diagnosis of Thrombotic Microangiopathies (TMA)
In many Thrombotic Microangiopathies (TMA) patients the onset of the disease is often happens very suddenly and result in severe illness. Patients are often hospitalized at the time of diagnosis. Diagnosis requires blood tests to confirm red blood cell destruction, the presence of schistocytes on blood smears, and organ damage that can be attributed to the TMA.
Typical organ damage includes very high blood pressure (malignant hypertension), kidney injury, abdominal pain, diarrhea, stroke, confusion, heart injury, and eye damage. Identifying the specific cause for TMA requires specialized blood and genetic testing to evaluate for the different causative diseases.
Some of this testing can take weeks to months to return. Since treatment must be initiated immediately, it is important to have a team of doctors experienced in the diagnosis and management of TMA.
Treatment of Thrombotic Microangiopathies (TMA)
Most TMA patients are treated with therapeutic plasma exchange, a procedure in which plasma is removed from the body and replaced with fresh donor plasma. This is effective therapy for TTP, and patients are usually treated with a combination of plasma exchange and immune suppression including corticosteroids.
For aHUS, patients are treated with an intravenous medication that blocks the complement system. For other diseases that cause TMA, the treatment focuses on managing the underlying disease. For example, infectious causes of TMA might be treated with antibiotics and supportive care.
Sometimes plasma exchange, immune suppression, or complement blocking therapies may be used to treat other causes of TMA. Patients with severe kidney injury may require dialysis. Some causes of TMA (e.g., aHUS and TTP) may be chronic, relapsing conditions that require ongoing therapy.
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