A patient with mixed type Evans syndrome: efficacy of rituximab treatment

Mixed type Evans syndrome is a very rare hematologic disease. Although mixed type Evans syndrome may initially respond well to steroids, this disease usually runs a chronic course with intermittent exacerbations. We describe here a 46-yr-old female with the steroid-refractory, mixed type Evans syndrome, and she had a prompt response to rituximab. She was diagnosed as having the mixed type Evans syndrome with the clinical features of symptomatic anemia, jaundice and thrombocytopenia. Prednisone therapy was commenced and her hemoglobin and platelet level returned to the normal. However, after 15 weeks, she relapsed with hemolytic anemia and thrombocytopenia. We started rituximab at the dose of 375 mg/m(2) once weekly for a total of 4 doses, which was well-tolerated and this induced the normalization of hemoglobin, bilirubin and lactic dehydrogenase, and there was also a significant increase of the platelet count.