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KMID : 1142020190540020131
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Blood Research
2019 Volume.54 No. 2 p.131 ~ p.136
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Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico
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Jaime-Perez Jose Carlos
Aguilar-Calderon Patrizia
Salazar-Cavazos Lorena
Leon Andres Gomez-De
Gomez-Almaguer David
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Abstract
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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±18months.
Conclusion: Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.
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KEYWORD
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Autoimmune hemolytic anemia, Low-dose rituximab, Refractory autoimmune hemolytic anemia, Steroids, Splenectomy, Immune cytopenia
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