We experienced a case of Evans syndrome with rapid and severe hemolysis due to mixed-type autoimmune hemolytic anemia (AIHA) and conducted an autoantibody assay for red blood cells (RBCs).
AIHA is characterized by autoantibody production against self RBCs, and is classified by the optimal temperature at which the antibodies react to the RBCs. Classifications of AIHA include warm-type AIHA, cold-type AIHA, paroxysmal cold hemoglobinuria, mixed-type AIHA, and drug-induced AIHA.
The patient was a 70-year-old woman who was admitted to our hospital on October 11, 2009 for general debility and numbness of the limbs. On admission, physical examination showed jaundice and anemia but enlargement of the liver and spleen were not observed. The blood exam showed a hemoglobin level of 4.7g/d
l, hematocrit of 13.7%, reticulocyte count of 47.6‰, and platelet count of 11,000/μ
l. Blood chemistry tests showed a total bilirubin level of 3.24mg/d
l, lactate dehydrogenase (LD) of 802U/
l, and hemoglobinuria positive. The direct antiglobulin test was positive for broad spectrum (4+), anti-C3bC3d (4+), and anti-IgG (4+). In the antibody test, cold agglutinin was detected but specificity of anti-I or anti-i was not observed, and had a titer of 1 : 128 by the saline method at 4°C and 1 : 512 by the albumin method at 4°C. Three days later, the patient died of multiple organ failure due to severe hemolytic anemia.
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