Durable response to combination therapy including staphylococcal protein A immunoadsorption in life-threatening refractory autoimmune hemolysis.
BACKGROUND
Few therapeutic options are available for severe, life-threatening, refractory autoimmune hemolytic anemia.


METHODS
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.


CONCLUSIONS
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.
Javeed
Mansoor
M
Department of Medicine, Milton S. Hershey Medical Center, Pennsylvania State University College of Medicine, Hershey 17033, USA.
Nifong
Thomas P
TP
Domen
Ronald E
RE
Rybka
Witold B
WB
D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D000789:Angina, Unstable / Q000209:etiology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D004351:Drug Resistance; D006388:Hemagglutinins / Q000097:blood / Q000276:immunology; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007565:Jaundice / Q000209:etiology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D008875:Middle Aged; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use*; D012074:Remission Induction; D013154:Spleen / Q000002:abnormalities; D013156:Splenectomy; D013205:Staphylococcal Protein A; D013575:Syncope / Q000209:etiology
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D006388:Hemagglutinins; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D013205:Staphylococcal Protein A; D011241:Prednisone; D008775:Methylprednisolone