Abstract
Cold agglutinin disease (CAD) is a rare form of autoimmune haemolytic anaemia, and because of its rareness, there is no standard treatment for CAD patients. We retrospectively analysed the response to rituximab-containing therapy in CAD patients at our hospital. All patients received rituximab-containing therapy for at least 1 month. A total of 16 patients (11 males and 5 females) were included. The median age at the onset of the disease was 63.5 years (range 41–79). Most patients had manifestations including anaemia (81.3%) or cold-induced circulatory symptoms (75.0%). The median haemoglobin level was 72 g/L (range 29–101), and the median cold agglutinin titre was 1,024 (range 64–2,048). Thirteen of 16 patients (81%) responded to the therapy. Responders achieved a median increase in haemoglobin levels of 45 g/L. Grade 3–4 neutropenia occurred in 3 patients (19%), but only 1 (6%) of them experienced infection. Anaphylaxis related to rituximab occurred in 1 patient. During follow-up, five patients experienced relapse, and two patients died. The estimated median progression-free survival was 36 months, and median overall survival was not yet reached. In conclusion, A rituximab-based therapy in accordance with individual patient characteristics may be a reasonable choice for CAD patients.
Highlights
Cold agglutinin disease (CAD) is a rare form of autoimmune haemolytic anaemia, and because of its rareness, there is no standard treatment for CAD patients
In 2017, Berentsen et al published their results in the use of bendamustine-rituximab therapy, which resulted in a similar response rate of 71%, with an even higher complete remission rate of 40% and sustained remissions, and the safety profile appeared more amenable than the fludarabine-rituximab c ombination[17]
Cold-induced circulatory symptoms were found in patients (75.0%), patients (81.3%) had symptoms related to anaemia such as fatigue and weakness, and 5 patients (31.3%) had symptoms pertinent to haemolysis such as jaundice and haemoglobinuria
Summary
Cold agglutinin disease (CAD) is a rare form of autoimmune haemolytic anaemia, and because of its rareness, there is no standard treatment for CAD patients. We retrospectively analysed the response to rituximab-containing therapy in CAD patients at our hospital. Most patients had manifestations including anaemia (81.3%) or cold-induced circulatory symptoms (75.0%). Most effective therapies have been directed at pathogenic B-cells, and the response rate and response duration need further improvement; bendamustine-rituximab therapy or rituximab monotherapy should be considered first-line treatments[15,18]. None of these studies have been confirmed sufficiently. Given the limitations in therapy choices for CAD, we have retrospectively analysed the efficacy of rituximabcontaining therapies in the treatment of CAD patients at our hospital
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