A Case Report of Refractory Warm Autoimmune Hemolytic Anemia Treated With Plasmapheresis and Rituximab

Abstract

A 30-year-old male presented with severe, warm autoimmune hemolysis 17 months subsequent to a matched, unrelated peripheral hematopoietic stem cell transplant. The patient responded poorly to conventional therapy with steroids and immunoglobulin, prompting the initiation of rituximab. On account of persistent, severe hemolysis, therapeutic plasma exchange was employed as a bridge until the rituximab therapy became effective. Immediately following plasmapheresis, the patient demonstrated clinical improvement followed by attenuation of the hemolysis and improved reticulocytosis. The hemoglobin concentration and reticulocyte index demonstrated further improvement following subsequent doses of rituximab and continued following the cessation of plasmapheresis. This case suggests the utility of plasmapheresis and rituximab in severe, life-threatening cases of warm autoimmune hemolytic anemia refractory to conventional therapy.

Role of therapeutic plasma exchanges in refractory severe warm autoimmune hemolytic anemia: Presentation of two case reports

“…In some cases, outcomes were not favorable, 10 at least in part because TPEs were not associated with immunosuppressive treatment. When TPEs are added in combination with rituximab, better results are reported 11 . These findings support the hypothesis that removing circulating autoantibodies with TPE might be not enough, and rituximab is essential to inhibit the production of autoantibodies.…”

“…When TPEs are added in combination with rituximab, better results are reported. 11 These findings support the hypothesis that removing circulating autoantibodies with TPE might be not enough, and rituximab is essential to inhibit the production of autoantibodies. The administration of IVIG avoids the hypogammaglobulinemia produced by TPEs 12,13 and the infective complications frequently reported in the past.…”

Systemic Lupus Erythematosus

“…440 Coombs-positive, autoimmune hemolytic anemia (AHA) occurs in approximately 10% of pSLE patients, though 30% to 40% may have a positive direct Coombs test without overt hemolysis. 437,[440][441][442][443] In the patient with SLE and anemia, other causes of anemia besides lupus need to be considered. 440,441 The first line of treatment for AHA is corticosteroids: oral for mild to moderate disease and intravenous for more severe or refractory anemia.…”

Life-Threatening Autoimmune Hemolytic Anemia Treated with Manual Whole Blood Exchange with Rapid Clinical Improvement

“…In patients with severe WAIHA, it may be extremely difficult to significantly reduce autoantibody levels due to high titers and biosynthetic rates. Not surprisingly, reports of plasmapheresis successfully moderating hemolysis involved multiple apheresis procedures in conjunction with aggressive immunosuppression [12,13]. Furthermore, the circulating free autoantibody present in plasma represents only a small fraction of the total intravascular autoantibodymost of which has already bound and sensitized RBC.…”

“…Alternative salvage therapies for resistant WAIHA, as single agents or in combination, are danazol, IVIG, alemtuzumab, cyclophosphamide, azathioprine, cyclosporine, mycophenolate mofetil, and vincristine-loaded platelets [1,[8][9][10]. Plasmapheresis has also been tried in severe AIHA as adjunct therapy, usually in combination with multi-agent immunosuppressive regimens [11][12][13].…”