Evans syndrome is defined at the combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). These diseases can present simultaneously or sequentially. Patients with Evans syndrome are thought to have a more severe disease process, more prone to bleeding, and to be more difficult to treat.
The classic clinical presentation of Evans syndrome is severe anemia and throm-bocytopenia with the cytopenias tending to be resistent to initial steroid therapy.
Children with Evans syndrome often have complex immunodeficiencies. In adults, Evans syndrome most often complicates other autoimmune diseases such as lupus. There are increasing reports of Evans sydrome occurring as a complication of T-cell lymphomas. Often the autoimmune disease can predate the lymphoma diagnosis by months or even years.
Several disease processes can present with both hemolysis and thrombocytope-nia (Table 11.3). Patients with congenital hemolytic syndromes are often thromb-ocytopenic, perhaps due to splenomegaly. Laboratory evidence of severe hemolysis and thrombocytopenia are the presenting signs of thrombotic microangiopathies.
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