Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review

Abstract

Until now, treatment of primary autoimmune hemolytic anemia of the warm type (wAIHA) is primarily based on immunosuppression. However, many patients do not respond adequately to treatment, and treated patients may develop severe side effects due to uncontrolled, mixed and/or long-lasting immunosuppression. Unfortunately, the newly used therapeutic monoclonal antibodies are unspecific and remain frequently ineffective. Thus, development of a specific therapy for AIHA is necessary. The ideal therapy would be the identification and elimination of the causative origin of autoimmunization and/or the correction or reprogramming of the dysregulated immune components. Blood transfusion is the most rapidly effective measure for patients who develop or may develop hypoxic anemia. Although some effort has been made to guide physicians on how to adequately treat patients with AIHA, a number of individual aspects should be considered prior to treatment. Based on my serological and clinical experience and the analysis of evidence-based studies, we remain far from any optimized therapeutic measures for all AIHA patients. Today, the old standard therapy using controlled steroid administration, with or without azathioprine or cyclophosphamide, is, when complemented with erythropoiesis-stimulating agents, still the most effective therapy in wAIHA. Rituximab or other monoclonal antibodies may be used instead of splenectomy in therapy-refractory patients.

How I treat autoimmune hemolytic anemia

“…The more commonly used treatments along with their corresponding dosing schedules are displayed in Table 1. [36][37][38][39][40][41][42] Patterns of care studies are not available, but single-agent rituximab is perhaps the most commonly used treatment in this setting and is our first choice after splenectomy relapse. In a meta-analysis of 21 studies that investigated rituximab, the overall response (OR) and CR rates were 79% and 42%, respectively.…”

Recurrent autoimmune hemolytic anemia in splenic marginal zone lymphoma

“…Steroid-resistant patients are treated with anti-CD20 therapies such as rituximab. Rituximab monotherapy is effective in SMZL with results similar to splenectomy and without the resultant post-splenectomy complications [8,9]. Studies comparing the overall survival of patients with SMZL treated with splenectomy and rituximab did not show any advantage of splenectomy over rituximab.…”

Treatment of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Presenting Simultaneously with Acquired Hemophilia and Warm Autoimmune Hemolytic Anemia

“…Hemolysis in CLL develops secondary to IgG antibodies produced by nonmalignant B-cell clones directed against red blood cell antigens, leading to clearance by the spleen and extravascular hemolysis (8). Th e fi rst-line treatment is steroids, 1 mg/kg/day, but this results in long-term remission in <15% to 20% of patients (9).…”

“…Rituximab results in response rates of 60% to 70%, but only 20% of patients achieve 5 years of remission (9).…”