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

Mansoor Javeed, Thomas P. Nifong, Ronald E. Domen, Witold B. Rybka

Research output: Contribution to journalArticle

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Abstract

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 degrees 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.

Original languageEnglish (US)
Pages (from-to)1217-1220
Number of pages4
JournalTransfusion
Volume42
Issue number9
DOIs
Publication statusPublished - Sep 2002

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All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Hematology

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