Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico

Abstract

Background Warm autoimmune hemolytic anemia (w-AIHA) is an uncommon disease with heterogeneous response to treatment. Steroids are the standard treatment at diagnosis, whereas rituximab has recently been recommended as the second-line therapy of choice. Our main objective was to document the response to treatment in patients with newly diagnosed w-AIHA, including the effectiveness of low-dose rituximab as frontline treatment and for refractory disease. Methods Patients with w-AIHA from 2002 to 2017 were included. Relapse-free survival (RFS), probability of maintained response (MR), and time-to-response were analyzed using the Kaplan–Meier method. Response was classified as complete, partial, and no response. Results We included 64 adults with w-AIHA (39 women and 25 men). The median age was 37 (16–77) years. Response rates to steroids alone were 76.7%, rituximab plus steroids, 100%; and cyclophosphamide, 80%. RFS with steroids at 6, 36, and 72 months was 86.3%, 65.1%, and 59.7%, respectively. Eighteen patients received rituximab at 100 mg/wk for 4 weeks plus high-dose dexamethasone as first-line therapy, with RFS at 6, 36, and 72 months of 92.3%, 58.7% and 44.1%, respectively. Eight patients refractory to several lines of therapy were treated with low-dose rituximab, and all achieved a response (three complete response and five partial response) at a median 16 days (95% confidence interval, 14.1–17.8), with a 75% probability of MR at 103 months; the mean MR was 81.93±18 months. Conclusion Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.

Efficacy of therapeutic plasma exchange for treatment of autoimmune hemolytic anemia: A systematic review and meta‐analysis of randomized controlled trials

“…Currently, corticosteroids are used as first‐line treatments against AIHA with rituximab serving as a second‐line therapy along with splenectomies 7,9,10 . Additional therapies, including intravenous immunoglobulins, high‐dose steroids, and therapeutic plasma exchange (TPE) are only recommended for patients with an acute onset of AIHA 7,9,11 .…”

“…However, due to the rarity of AIHA, there is a lack of randomized controlled trials (RCTs) investigating the efficacy and safety of the aforementioned regimens in English literature sources 7,12,13 . To date, there is no evidence‐based treatment strategy for AIHA; clinical management of AIHA is largely dependent upon physician experience and patient characteristics 7,9,14 …”

Rituximab Use in Warm and Cold Autoimmune Hemolytic Anemia

“…Subsequently, efficacy was also demonstrated in autoimmune disorders such as rheumatoid arthritis (RA); systemic lupus erythematosus; multiple sclerosis; and several hematologic autoimmune diseases such as immune thrombocytopenic purpura (ITP), acquired hemophilia, thrombotic thrombocytopenic purpura, and AIHA [ 41 , 42 , 43 , 44 , 45 ]. In AIHA, rituximab demonstrated efficacy in the first line as well as relapsed setting [ 43 , 46 , 47 , 48 , 49 , 50 , 51 , 52 ] in primary as well as secondary forms [ 49 , 53 ] of wAHA, cold agglutinin disease, and secondary cold agglutinin syndrome.…”

Rituximab use in Warm and Cold Autoimmune Hemolytic Anemia

“…Subsequently, efficacy was also demonstrated in autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis, and several hematologic autoimmune diseases such as immune thrombocytopenic purpura (ITP), acquired hemophilia, thrombotic thrombocytopenic purpura, and AIHA [41][42][43][44][45]. In AIHA rituximab demonstrated efficacy in the first line as well as relapsed setting [43,[46][47][48][49][50][51][52] in primary as well as secondary forms [49,53] of wAHA, cold agglutinin disease, and secondary cold agglutinin syndrome.…”

“…In steroid refractory patients with autoimmune cytopenia low dose rituximab in combination with anti-CD52 MoAb alemtuzumab produced responses in 100% of patients with complete response in 58% [118]. In wAIHA, a retrospective analysis of 64 adults reported response rates of 76.7% to steroids alone and 100% to rituximab at 100 mg/wk for 4 weeks in combination with high-dose dexamethasone [52].…”