| Abstract | | Background Cold agglutinin disease is an uncommon hemolytic anemia associated sometimes with low-grade B-cell lymphoproliferative disorders. The disease results from increased production of anti erythrocyte antibodies. Standard treatment is mostly unsuccesful. Patient and methods A 76-years-old patient is presented who was diagnosed with hemolytic anemia caused by cold agglutinins. Later a bone marrow located B cell CD 20 non Hodgkin lymphoma was diagnosed. Standard treatment was unsuccesful, so she received rituximab. After 3 cycles the signs of hemolysis disappeared and the hemoglobin level normalized. The patient is treated every 3 months with rituximab and is in complete remission. Conclusions Treatment with rituximab is used also for treating immune cytopenias. There are some reports of treating cold agglutinin anemia, where standard treatment is mostly without success. As this is a rare disease only some small studies and case reports have demonstrated the beneficial effect of rituximab on this type of anemia. Despite its high price, rituximab has numerous advantages: immediate and long lasting effect and relatively few side effects.
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