EVANS SYNDROME
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EVANS SYNDROME
- 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.
A person 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 person will develop one blood disorder first and then another one later. Sometimes, a person will develop both disorders at the same time, but this is less common.
A person must have two of these conditions for a doctor to diagnose Evans syndrome.
Experts do not know how many people get Evans syndrome. of adults with Evans syndrome found that it may affect up to 21.3 of every million people.According to the NORD, the incidence and prevalence of Evans syndrome are unknown. The organization describe this condition as rare.
- 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.
A person 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 person will develop one blood disorder first and then another one later. Sometimes, a person will develop both disorders at the same time, but this is less common.
A person must have two of these conditions for a doctor to diagnose Evans syndrome.
Symptoms
Due to the immune system attack that Evans syndrome causes, people 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 people have periods of remission during which symptoms of the disease go away. Remission may happen after successful treatment. However, people may also have periods when symptoms get worse.
Due to the immune system attack that Evans syndrome causes, people 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 people have periods of remission during which symptoms of the disease go away. Remission may happen after successful treatment. However, people 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:
- fatigue
- pale skin
- dizziness or lightheadedness
- shortness of breath
- rapid heartbeat
- yellowing of the skin and eyes (jaundice)
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:
- fatigue
- pale skin
- dizziness or lightheadedness
- shortness of breath
- rapid heartbeat
- 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 person may notice:
- tiny purple or red spots on the skin (petechiae)
- purple spots on the skin, which may appear raised (purpura)
- larger purple blotches on the skin (ecchymosis)
- skin that bruises easily
Low levels of platelets may cause bleeding under the skin because the blood cannot clot properly.
If this occurs, a person may notice:
- tiny purple or red spots on the skin (petechiae)
- purple spots on the skin, which may appear raised (purpura)
- larger purple blotches on the skin (ecchymosis)
- skin that bruises easily
Low neutrophils
People who have low levels of neutrophils are more likely to get infections. Their symptoms may include:
- a fever
- feeling unwell
- sores or ulcers inside the mouth
- frequent illnesses
People with Evans syndrome may also have an enlarged spleen, liver, or lymph nodes.
A person may not notice these signs, but a doctor can identify them during an exam or on an imaging test.
Treatment
People who have low levels of neutrophils are more likely to get infections. Their symptoms may include:
- a fever
- feeling unwell
- sores or ulcers inside the mouth
- frequent illnesses
People with Evans syndrome may also have an enlarged spleen, liver, or lymph nodes.
A person may not notice these signs, but a doctor can identify them during an exam or on an imaging test.
Treatment
There is a long list of possible treatments. Treatments are directed at the specific blood cell affected and whether you have any symptoms (active bleeding, shortness of breath, elevated heart rate, infection):
- Steroids: Medications like prednisone have been used for years in various autoimmune disorders. They are the first line treatment for AIHA and are also used in ITP. Unfortunately, if you have Evans Syndrome, you may require steroids for long periods of time which may result in other issues like high blood pressure (hypertension) and elevated blood sugars (diabetes). For this reason, your physician may look for alternative therapies.
- Intravenous Immunoglobulin (IVIG): IVIG is a first-line treatment for ITP. Essentially, the IVIG distracts your immune system temporarily so that the platelets are not destroyed as rapidly. IVIG is not as effective in AIHA or AIN.
- Splenectomy: The spleen is the main location of the destruction of the red blood cells, platelets, and neutrophils in Evans Syndrome. In some patients, surgical removal of the spleen might improve blood counts, but this may be only temporary.
- Rituximab: Rituximab is a medication called a monoclonal antibody. It reduces your B-lymphocytes (a white blood cell that makes antibodies), which can improve your blood counts.
- G-CSF (filgrastim): G-CSF is s a medication used to stimulate the bone marrow to make more neutrophils. It is sometimes used to increase the neutrophil count in AIN, particularly if you have an infection.
- Immunosuppressive Medications: These medications inhibit the immune system. These would include medications like mycophenolate mofitel (MMF), azathioprine (Imuran), tacrolimus (Prograf).
Although patients may respond to individual treatments with improvement in blood counts, this response if often temporary requiring additional treatments.
Medical Coding, Medical Sribing, Medical Transcription, Digital Marketing, OET training
There is a long list of possible treatments. Treatments are directed at the specific blood cell affected and whether you have any symptoms (active bleeding, shortness of breath, elevated heart rate, infection):
- Steroids: Medications like prednisone have been used for years in various autoimmune disorders. They are the first line treatment for AIHA and are also used in ITP. Unfortunately, if you have Evans Syndrome, you may require steroids for long periods of time which may result in other issues like high blood pressure (hypertension) and elevated blood sugars (diabetes). For this reason, your physician may look for alternative therapies.
- Intravenous Immunoglobulin (IVIG): IVIG is a first-line treatment for ITP. Essentially, the IVIG distracts your immune system temporarily so that the platelets are not destroyed as rapidly. IVIG is not as effective in AIHA or AIN.
- Splenectomy: The spleen is the main location of the destruction of the red blood cells, platelets, and neutrophils in Evans Syndrome. In some patients, surgical removal of the spleen might improve blood counts, but this may be only temporary.
- Rituximab: Rituximab is a medication called a monoclonal antibody. It reduces your B-lymphocytes (a white blood cell that makes antibodies), which can improve your blood counts.
- G-CSF (filgrastim): G-CSF is s a medication used to stimulate the bone marrow to make more neutrophils. It is sometimes used to increase the neutrophil count in AIN, particularly if you have an infection.
- Immunosuppressive Medications: These medications inhibit the immune system. These would include medications like mycophenolate mofitel (MMF), azathioprine (Imuran), tacrolimus (Prograf).
Although patients may respond to individual treatments with improvement in blood counts, this response if often temporary requiring additional treatments.
Medical Coding, Medical Sribing, Medical Transcription, Digital Marketing, OET training
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