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Published on: November 26, 2018
[Autoimmune hemolytic anemia associated with B-cell chronic lymphoproliferative disorders]
Yun Zhuang1, Lei Fan, Yun-Feng Shen
1Department of Hematology, Wuxi People Hospital Affiliated of Nanjing Medical University, Wuxi, Jiangsu Province, China.
Insights
Autoimmune hemolytic anemia (AIHA) frequently complicates B-cell chronic lymphoproliferative disorders (B-CLPD). Corticosteroids offer initial response, but rituximab shows efficacy in managing these challenging cases.
Area of Science:
- Hematology
- Oncology
- Immunology
Context:
- B-cell chronic lymphoproliferative disorders (B-CLPD) can present with autoimmune hemolytic anemia (AIHA).
- Understanding the clinical spectrum and treatment outcomes of B-CLPD with AIHA is crucial for patient management.
Purpose:
- To investigate the clinical characteristics, treatment strategies, and outcomes of patients with B-cell chronic lymphoproliferative disorders complicated by autoimmune hemolytic anemia.
Summary:
- Retrospective analysis of 14 patients with B-CLPD and AIHA revealed chronic lymphocytic leukemia (9 cases) and lymphoma (5 cases) as primary diagnoses.
- All patients showed a positive Coombs test; corticosteroids provided initial response, but rituximab-based immunochemotherapy demonstrated superior long-term efficacy with a high overall response rate (100%) and complete remission (78.6%).
- While corticosteroid monotherapy had limitations, rituximab, particularly in combination, proved effective in managing relapsed hemolysis, with only one patient relapsing after rituximab treatment.
Impact:
- AIHA is a common complication of B-CLPD, occurring across disease stages.
- Corticosteroids are effective for initial AIHA treatment in B-CLPD, though long-term remission rates are lower.
- Rituximab is confirmed as an effective therapeutic option for B-CLPD complicated by AIHA, improving patient outcomes.
Abstract:
This study was purpose to investigate the clinical characteristics of B-cell chronic lymphoproliferative disorders (B-CLPD) complicated by autoimmune hemolytic anemia (AIHA) so as to improve the understanding of this disease. The clinical characteristics, laboratory data, therapy and outcome of 14 patients suffering from B-CLPD complicated by AIHA were retrospectively analyzed in Wuxi People Hospital and the First Affiliated Hospital of Nanjing Medical University from 2000 to 2012. The results showed that 9 cases of the 14 patients were patients with chronic lymphocytic leukemia (CLL), 5 cases were patients with lymphoma, at time of hemolysis the median level of hemoglobin was 61 (33 - 84)g/L, the median ratio of reticulocytes was 12.0 (3.1 - 35.0)%, the positive rate of Coombs test was 100%. 1 case received corticosteroid alone, 5 cases were treated with chemotherapy combined with corticosteroid, 8 cases were treated with immunochemotherapy rituximab combined with corticosteroid. Overall response rate was 100%, in which CR was 78.6% (11/14), PR was 21.4% (3/14). The follow-up for these patients were performed to now, 35.7% (5/14) patients relapsed with hemolysis again, but they showed therapeutic response to treatment with above-mentioned therapy. From patients treated with rituximab alone, only 1 patient relapsed. Among 14 patients, 6 cases died, 1 case was lost, the other cases are still alive. It is concluded that the AIHA is the commonest complication of B-CLPD, it can be observed at different stages of B-CLPD, the treatment with corticosteroids can give well therapeutic effect for these patients, but the long time CR is lower, the rituximab has been confirmed to be effective for B-CLPD complicated by AIHA.
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