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Evans syndrome is
a rare autoimmune disorder in which the immune system mistakenly attacks
red blood cells and platelets, causing autoimmune haemolytic
anaemia and immune thrombocytopenia, either simultaneously or at
different times. In some patients, immune neutropenia may also occur.
The condition can develop independently or alongside other autoimmune
diseases, immune deficiencies, infections, or lymphoproliferative
disorders.
Symptoms depend on the
affected blood cells. Anaemia may cause fatigue, weakness, pale
skin, shortness of breath, dizziness, or rapid heartbeat. Low
platelet levels can lead to easy bruising, petechiae, nosebleeds, or
excessive bleeding. Diagnosis involves blood counts, blood film examination, reticulocyte testing, bilirubin and lactate dehydrogenase
measurements, and a direct antiglobulin test. Other causes of haemolysis
and thrombocytopenia must be excluded.
Treatment aims to control
abnormal immune activity and prevent complications. Corticosteroids are
commonly used as initial therapy, while intravenous immunoglobulin may be
considered when a rapid increase in platelet levels is needed. Relapsing or
treatment-resistant cases may require rituximab, immunosuppressive medicines,
or other specialist therapies. Regular monitoring of blood counts is important
because Evans syndrome often follows a chronic, relapsing course.
Early diagnosis and individualized treatment can help reduce bleeding,
anaemia, and other complications.