What is idiopathic, autoimmune hemolyticanemia?
An autoimmune form of hemolytic anemia called idiopathic autoimmune is also known as autoimmune hemolytic. Autoimmune hemolytic aemia (AIHA), is a rare, but severe blood disorder. These conditions occur when the body damages red blood cells faster than it produces. If the cause of the condition is not known, it is considered idiopathic.
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Autoimmune diseases are diseases that attack the body. Your immune system makes antibodies to fight foreign invaders like bacteria and viruses. Your body may mistakenly produce antibodies that attack your body in the case of autoimmune diseases. Your body creates antibodies to destroy red blood cells in AIHA.
Who is at greatest risk?
Around 50% of all AIHA cases involve idiopathic causes. AIHA can happen at any time in your life. It can also develop gradually or suddenly. It is more common in women.
AIHA that isn’t idiopathic means it wasn’t caused by an underlying condition or medication. Idiopathic AIHA is not a sign of underlying disease. Individuals with idiopathic AIHA might have abnormal blood tests results but no symptoms.
Idiopathic AIHA symptoms
Sudden-onset idiopathic AIHA can cause weakness and shortness of breath. Other cases, such as chronic, progressive AIHA, symptoms may not be apparent. Both cases may present with one or more of these symptoms:
- Increasing weakness
- Breathing difficulty
- rapid heartbeat
- Skin that is pale or yellow
- muscle pain
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- nausea
- vomiting
- Dark-colored urine
- A headache
- Abdominal discomfort
- Bloating
- diarrhea
Diagnosing idiopathic AIHA
If you suspect that you may have AIHA, your doctor will discuss your symptoms with you. Before diagnosing you with AIHA, they will need to rule out any medications or other conditions that could be the cause.
Your doctor will first ask about your medical history. If your symptoms are severe, they will likely admit you to the hospital immediately for testing and monitoring. Discolored skin, severe anemia and urine problems are all examples of serious conditions. You may be referred to a blood specialist or a hematologist.
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To confirm AIHA, you will need to undergo a series of blood tests. Some tests will measure your body’s red cell count. Your red blood cell count will be lower if you have AIHA. The blood will also be tested for other substances. A high ratio of mature to immature red blood cells in blood tests may indicate AIHA. The body may be trying to replace mature red blood cell loss too quickly by generating a high level of immature blood cells.
IAIHA Treatment Options
Because of the acute nature of sudden onset idiopathic AIHA, people who are suspected will be admitted to hospital immediately. Sometimes chronic cases can come and go with no explanation. The condition can improve with or without treatment.
If you have diabetes, your doctor will closely monitor your blood sugar levels. Treatment for diabetes can lead to serious infections.
Steroids
Prednisone is the first-line treatment. They can increase red blood cell count. To ensure that steroids are effective, your doctor will closely monitor you. Your doctor will gradually wean you off the steroids once your condition is in remission. Supplements may be required for patients with AIHA who are undergoing steroid treatment. These supplements could include:
- bisphosphonates
- Vitamin D
- calcium
- Folic acid
- Surgery
If the steroids are not working, your doctor might recommend that you have the spleen removed surgically. The destruction of red blood cells can be reversed by removing the spleen. This is called a splenectomy. A splenectomy can result in a partial or complete remission of their AIHA. People with the idiopathic type are more likely to experience the best results.
Anti-inflammatory drugs
There are also immune-suppressing medications, like azathioprine or cyclophosphamide. These drugs can be very effective for patients who haven’t responded to steroids treatment or are not candidates for surgery.
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Rituximab is sometimes preferred to traditional immuno-suppressing drugs in certain cases. Rituximab, an antibody that targets specific proteins on immune system cells, is known as an anti-inflammatory drug.