Bay Biosciences provides high quality, clinical grade bio-samples, biopsy samples, cryogenically preserved sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Evans Syndrome.
The sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The Evans Syndrome bio-specimens are collected from unique patients diagnosed with Evans Syndrome and are provided to a valued pharmaceutical customer for translational research, genomics, proteomics and biomarker research, drug discovery and development.
Evans Syndrome Overview
Evans Syndrome is a rare autoimmune disease that causes an individual’s immune system attacks and destroy their own red blood cells and platelets. It can cause low blood cell levels and anemia, which may lead to a variety of symptoms. Evans syndrome can include immune neutropenia (abnormally low concentration of neutrophils type of WBC’s). These immune cytopenias (reduction in the number of mature blood cells) may occur simultaneously or sequentially.
Its overall phenotype resembles a combination of autoimmune hemolytic anemia and immune thrombocytopenic purpura. Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.
Evans syndrome is a rare combination of two or more of the following autoimmune blood disorders:
- Autoimmune Hemolytic Anemia (AIHA), in which the immune system destroys red blood cells. Red blood cells are responsible for carrying oxygen to the body’s organs and tissues.
- Immune Thrombocytopenia (ITP), in which the immune system attacks platelets. Platelets help stop bleeding by forming blood clots.
- Autoimmune Neutropenia (AIN), which causes the immune system to attack neutrophils. Neutrophils are white blood cells that help with healing tissues and fighting infections.
Patients with Evans syndrome will have AIHA along with ITP or AIN. A combination of AIHA with ITP is more common, according to the National Organization for Rare Disorders (NORD). Usually, a patient will develop one blood disorder first and then another one later. Sometimes, a patient will develop both disorders at the same time, but this is less common. Patients must have two of these conditions for a doctor to diagnose Evans syndrome. Experts do not know how many people get Evans syndrome. According to the NORD, the incidence and prevalence of Evans syndrome are unknown, the organization describe this condition as rare.
Evans Syndrome Symptoms
Due to the immune system attack that Evans syndrome causes, patients with this condition have abnormally low levels of blood cells, doctors may refer to this as cytopenia. Cytopenia causes a variety of symptoms that vary from person to person. The severity of the disease and the specific blood cells that the disease involves will determine what symptoms a person develops. Some patients have periods of remission during which symptoms of the disease go away. Remission may happen after successful treatment. However, Evans syndrome patients may also have periods when symptoms get worse.
Anemia Symptoms
Low levels of red blood cells due to Evans disease may cause anemia. Anemia means that the body does not have enough red blood cells to get oxygen to the organs and tissues.
Anemia may cause the following symptoms:
- Dizziness or Lightheadedness
- Fatigue
- Pale Skin
- Rapid Heartbeat
- Shortness of breath
- Yellowing of the skin and eyes (Jaundice)
Low Platelets
Low levels of platelets may cause bleeding under the skin because the blood cannot clot properly.
If this occurs, a patient may notice:
- Large purple blotches on the skin (Ecchymosis)
- Purple spots on the skin which may appear raised (Purpura)
- Skin that bruises easily
- Tiny purple or red spots on the skin (Petechiae)
Low Neutrophils
Patients who have low levels of neutrophils are more likely to get infections. People with Evans syndrome may also have an enlarged spleen, liver, or lymph nodes.
The symptoms may include:
- Fever
- Feeling Unwell
- Frequent Illnesses
- Sores or ulcers inside the mouth
Evans Syndrome Causes
Researchers do not know why some people get Evans syndrome, Evans syndrome does not usually appear to run in families. A healthy immune system consists of cells, tissues, and organs that defend the body against germs, diseases, and invaders. When the immune system senses an invader, it makes proteins called antibodies that destroy the foreign substance. In Evans syndrome, the immune system treats blood cells as invaders, making antibodies that harm them. Certain triggers may lead to Evans syndrome, possible triggers include illnesses, infections, and other health conditions.
Evans Syndrome Diagnosis
There is no specific test that will diagnose Evans syndrome. Instead, a doctor will run a variety of tests to rule out other health issues. Some of the tests that doctors may use to confirm a diagnosis include:
- Complete blood count (CBC), which measures a person’s number of blood cells
- Direct or indirect Coombs test, which looks for antibodies against red blood cells (positive in AIHA)
- Bone marrow biopsy
- CT scans of the chest, abdomen, and pelvis
A doctor usually diagnoses Evans syndrome if the person has both AIHA and ITP, even if they do not develop these disorders at the same time.
Evans Syndrome Treatment
There is no cure for Evans syndrome, so treatment focuses on relieving symptoms and increasing blood cell counts. Treatment for Evans syndrome varies from person to person. The most suitable treatment will depend on the person’s:
- Age
- Blood Cell Count
- Health Status
- Symptoms
Corticosteroids
One of the most common treatments for Evans syndrome is corticosteroids. These medications can help lower the activity of the immune system. However, corticosteroids can have many side effects, including:
- High Blood Pressure
- High Blood Sugar
- Mood Swings
- Weight Gain
Intravenous Immunoglobulin
Intravenous Immunoglobulin (IVIg) is a treatment that contains antibodies from healthy donors. A patient receives this treatment intravenously, which means through an injection into the veins. IVIg can help prevent the immune system from attacking the body.
Immunosuppressive Drugs
Immunosuppressive medications work by lowering the level of activity of the immune system. Possible side effects include a higher risk of getting illnesses and infections.
Spleen Removal
In some cases, treatment may involve surgically removing the spleen in a procedure called a splenectomy. However, patients with Evans syndrome have mixed responses to this procedure, so it is hard to determine whether it helps with symptoms. The surgical procedures also carries additional risks. Some researchers suggest that the risks of splenectomy outweigh its benefits, especially because newer treatments, such as rituximab, are now available.
Hematopoietic Stem Cell Transplantation
A stem cell transplant could help reset the immune system and provide a potential cure for Evans syndrome. The treatment requires a donor to provide stem cells. The donor will usually be a close family member who is a proper match with the patient. However, stem cell treatment may not work for everyone, and no large studies have yet proved that it is effective.
Detailed clinical data, CT scans, coombs test, bone marrow biopsy, blood analysis, elevated biomarker levels, genetic and metabolic information, histopathological findings, annotations associated with the Evans Syndrome patient’s specimens is provided to a valued customer for research, development and drug discovery. The Evans Syndrome sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patients peripheral whole-blood using customized processing protocols provided by the researcher.
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