Blood Res.  2019 Jun;54(2):131-136. 10.5045/br.2019.54.2.131.

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

• Jaime-Pérez JC
• Aguilar-Calderón P
• Salazar-Cavazos L
• Gómez-De León A
• Gómez-Almaguer D

• KMID: 2451014
• DOI: http://doi.org/10.5045/br.2019.54.2.131

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.

Keyword

Autoimmune hemolytic anemia; Low-dose rituximab; Refractory autoimmune hemolytic anemia; Steroids; Splenectomy; Immune cytopenia

MeSH Terms

Adult
Anemia, Hemolytic, Autoimmune*
Cyclophosphamide
Dexamethasone
Diagnosis
Female
Humans
Methods
Mexico*
Rituximab
Splenectomy
Steroids
Cyclophosphamide
Dexamethasone
Rituximab
Steroids

Figure

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  Fig. 1 Comparison between hemoglobin levels at diagnosis, after treatment and at last follow-up in 64 patients with w-AIHA, including median and ranges.

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  Fig. 2 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.

• 

  Fig. 3 Time to response (days) in 8 patients with refractory warm autoimmune hemolytic anemia treated with rituximab at 100 mg/wk/4 weeks (A). Maintained response (MR) probability in 6 patients with refractory w-AIHA treated with rituximab at 100 mg/wk/4 weeks (B). Two patients were excluded because MR was less than 6 months.

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