What is Evans syndrome?
Evans syndrome is a rare condition characterized by autoimmune destruction of red blood cells (RBCs), platelets, and sometimes white blood cells (WBCs), particularly neutrophils. This results in autoimmune hemolytic anemia (AIHA), idiopathic thrombocytopenic purpura (ITP), and neutropenia.
This can progress to severe life-threatening complications. Evans syndrome can occur as a primary disorder or as a secondary disorder along with lymphoproliferative disorders or other autoimmune disorders.
Signs and symptoms
Signs and symptoms of Evans syndrome vary from person to person and include:
- Anemia symptoms such as paleness, fatigue, shortness of breath, lightheadedness, jaundice, and fast heartbeat.
- Thrombocytopenia symptoms such as petechiae (red or purple spots on the skin), ecchymosis (purple discoloration of the skin due to rupturing of blood vessels in the subcutaneous tissue), and purpura.
- Neutropenia causes symptoms such as recurrent and frequent infections, ulcers, malaise, and overall poor health.
- Enlargement of lymph nodes.
- Enlargement of spleen and liver.
If left untreated, this can progress to life-threatening conditions such as sepsis, heart failure, and hemorrhage.
Cause
The exact cause of Evans syndrome is not known. It is actually an autoimmune disorder and manifests when the immune system produces antibodies that are directed against the red blood cells, platelets, and neutrophils.
Diagnosis
Diagnosis of Evans syndrome can be carried out by complete and detailed patient history, proper clinical evaluation, identification of the signs and symptoms, and by running blood tests in the laboratory. There isn’t a single specific blood test that can help diagnose Evans syndrome but a diagnosis can be made when thrombocytopenia and autoimmune hemolytic anemia occur in the same patient at the same time.
- Lab tests should include a complete blood count (CBC) which can help find out the low levels of red blood cells, neutrophils, and platelets.
- Another test called the direct antiglobulin test (DAT) is carried out to test whether a specific antibody is present in higher than normal amounts or not.
- Antibody assays
- CT scans of the pelvis, chest, and abdomen
Treatment
Treatment of Evans syndrome can be quite challenging and in general, there is no cure for Evans syndrome as of yet. Treatment is mostly symptomatic and includes:
- Corticosteroids such as prednisolone for immunosuppression and prevent the formation of autoantibodies.
- Intravenous immunoglobulin therapy (IVIg) involves the injection directly into the vein of the patient of antibodies taken from a healthy individual, in an effort to modify the immune system.
- Splenectomy (removal of the spleen) can be done in some individuals who do not respond to other therapeutic procedures.
- Blood or platelet transfusions in acute cases.
- Rituximab is found to be effective in the treatment of Evans syndrome as it a monoclonal antibody (created artificially in the lab). This is a new therapy and it is advantageous in the sense that it prevents serious immunosuppression and also avoids side effects associated with other immunosuppressive drugs.
- Another drug called mycophenolate mofetil is administered in patients who do not respond to corticosteroids or intravenous immunoglobulin therapy.
