Blood Res 2019; 54(2):
Published online June 30, 2019
https://doi.org/10.5045/br.2019.54.2.131
© The Korean Society of Hematology
Correspondence to : José Carlos Jaime-Pérez, M.D., Ph.D.
Hospital Universitario Dr. José E. González, Hematología, Edificio “Dr. Rodrigo Barragán Villarreal” 2o piso Ave. Maderoy Ave. Gonzalitos s/n, Colonia Mitras Centro, CP 64460 Monterrey, N.L. Mexico
E-mail: carjaime@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
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.
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.
Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.
Keywords Autoimmune hemolytic anemia, Low-dose rituximab, Refractory autoimmune hemolytic anemia, Steroids, Splenectomy, Immune cytopenia
Blood Res 2019; 54(2): 131-136
Published online June 30, 2019 https://doi.org/10.5045/br.2019.54.2.131
Copyright © The Korean Society of Hematology.
José Carlos Jaime-Pérez, Patrizia Aguilar-Calderón, Lorena Salazar-Cavazos, Andrés Gómez-De León, David Gómez-Almaguer
Department of Hematology, Internal Medicine Division, Dr. José E. González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Mexico
Correspondence to:José Carlos Jaime-Pérez, M.D., Ph.D.
Hospital Universitario Dr. José E. González, Hematología, Edificio “Dr. Rodrigo Barragán Villarreal” 2o piso Ave. Maderoy Ave. Gonzalitos s/n, Colonia Mitras Centro, CP 64460 Monterrey, N.L. Mexico
E-mail: carjaime@hotmail.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
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.
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.
Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.
Keywords: Autoimmune hemolytic anemia, Low-dose rituximab, Refractory autoimmune hemolytic anemia, Steroids, Splenectomy, Immune cytopenia
Comparison between hemoglobin levels at diagnosis, after treatment and at last follow-up in 64 patients with w-AIHA, including median and ranges.
Relapse-free survival in 54 patients who achieve a response treated with steroids only or rituximab at 100 mg/wk/4 weeks plus high doses of dexamethasone.
Time to response (days) in 8 patients with refractory warm autoimmune hemolytic anemia treated with rituximab at 100 mg/wk/4 weeks
Table 1 . Comparison of important characteristics in 64 adults with warm autoimmune hemolytic anemia treated with steroids only or low-dose rituximab plus high-dose dexamethasone as first-line therapy..
Values presented in the table are N (%) or value (range)..
Abbreviations: HDD, high doses of dexamethasone; LDH, lactic dehydrogenase; RBCs, red blood cells..
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Comparison between hemoglobin levels at diagnosis, after treatment and at last follow-up in 64 patients with w-AIHA, including median and ranges.
|@|~(^,^)~|@|Relapse-free survival in 54 patients who achieve a response treated with steroids only or rituximab at 100 mg/wk/4 weeks plus high doses of dexamethasone.
|@|~(^,^)~|@|Time to response (days) in 8 patients with refractory warm autoimmune hemolytic anemia treated with rituximab at 100 mg/wk/4 weeks