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Case Report: Autoimmune Haemolytic Anaemia Responds to Treatment With Rituximab

A patient with relapsed autoimmune haemolytic anaemia (AIHA) and a history of Evans syndrome responded completely to treatment with rituximab, according to a new case report.

Clinical manifestations of Evans syndrome include AIHA and immune thrombocytopaenia. Treatment options include steroids, splenectomy, and intravenous immunoglobulin but, often, the symptoms become refractory to treatment.

According to the authors, corticosteroids are often effective as a first-line treatment for AIHA; however, there is no consensus regarding their use for second-line treatment. By comparison, immunosuppressive therapy has not consistently been shown to be effective in the treatment of refractory or relapsing AIHA.

Rituximab is an antibody to the CD20 antigen found on the surface of B lymphocytes. Recent reports have suggested that rituximab has activity against cold agglutinin disease and AIHA in patients with lymphoma, as well as in systemic lupus erythematosus.

In their case report, Anat Galor, MD, and Timothy O'Brien, MD, with the Case Western Reserve University School of Medicine, Cleveland, Ohio, United States, describe the case of a 43-year-old white male with a history of immune thrombocytopaenia, splenectomy, juvenile rheumatoid arthritis, and adult-onset Stills disease.

The patient had previously been treated with steroids, splenectomy, intravenous immunoglobulin, plasmapheresis, staphylococcal Protein A immunoadsorption, and chemotherapy with cytoxan and vincristine.

Rituximab was administered weekly at 375 mg/m² for 4 doses. The drug was well tolerated, and the patient had only mild flu-like symptoms after the second dose. The patient's haematocrit increased and his lactate dehydrogenase (LDH) decreased. Platelets remained normal through the AIHA exacerbation, the authors note.

Nine months after his initial treatment, the patient was still in remission.

"Recent reports suggest that rituximab may be an effective treatment for chronic immune thrombocytopenia and steroid refractory AIHA associated with lymphoproliferative disorders," Drs. Galor and O'Brien note.

"We report a complete response to this agent in a patient with AIHA but without such an underlying disorder. Larger studies will need to be conducted to confirm this finding," they suggest.

Int J Hematol 2003;78:335-336. "Rituximab treatment for relapsed autoimmune hemolytic anemia in Evans syndrome"

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