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Durable response to combination therapy including staphylococcal protein A immunoadsorption in life‐threatening refractory autoimmune hemolysis
Author(s) -
Javeed Mansoor,
Nifong Thomas P.,
Domen Ronald E.,
Rybka Witold B.
Publication year - 2002
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1046/j.1537-2995.2002.00193.x
Subject(s) - medicine , immunoadsorption , autoimmune hemolytic anemia , cyclophosphamide , plasmapheresis , hemolysis , refractory (planetary science) , apheresis , anemia , gastroenterology , surgery , immunology , antibody , chemotherapy , platelet , physics , astrobiology
BACKGROUND : Few therapeutic options are available for severe, life‐threatening, refractory autoimmune hemolytic anemia. CASE REPORT : A 53‐year‐old 110‐kg man was seen with acute onset of symptomatic severe anemia with syncope, unstable angina, and jaundice. His nadir Hct was 8.3 percent with a peak total bilirubin 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°C. Treatment with high‐dose corticosteroids and IVIG was instituted. An accessory spleen measuring 2 cm was identified and surgically removed, but the patient continued to have intense hemolysis. Cyclophosphamide 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. Cyclophosphamide 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 hemolysis can be a life‐threatening event. The patient 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.