Durable response to combination therapy including staphylococcal protein A immunoadsorption in life-threatening refractory autoimmune XXXX .

@entity1 @entity417 @entity1087 @entity648 @entity786 @entity1163 @entity1459 @entity5714 @entity2142 @entity288
BACKGROUND: Few therapeutic options are available for severe, life-threatening, refractory autoimmune @entity5714 . CASE REPORT: A 53-year-old 110-kg @entity1 was seen with acute onset of symptomatic severe @entity417 with @entity1163 , @entity1459 , and @entity1087 . His nadir Hct was 8.3 percent with a peak total @entity648 of 44 mg per dL. The DAT was positive but the IAT was negative. Elution studies demonstrated an IgG pan-agglutinin antibody reactive at 37 degrees C. Treatment with high-dose @entity288 and IVIG was instituted. An accessory spleen measuring 2 cm was identified and surgically removed, but the @entity1 continued to have intense @entity2142 . @entity786 at 200 mg per day was started. Apheresis with a staphylococcal protein A immunoadsorption column (Prosorba, Cypress Bioscience, Inc.) was initiated on Day 18 and was performed twice weekly for a total of six treatments. @entity786 was continued for a total of 14 days. His transfusion requirement ceased by the third immunoadsorption treatment. Forty units of RBCs were required over 23 days in an attempt to maintain a Hct greater than or equal to 15 percent. CONCLUSION: Refractory autoimmune @entity2142 can be a life-threatening event. The @entity1 did not achieve a response until after several different therapeutic modalities were instituted, including plasmapheresis with a staphylococcal protein A column (Prosorba). A complete response continues to be durable for more than 1 year after therapy.
