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ASH 2025 | Real-world outcomes of patients with cold agglutinin disease
In this video, Jahanzaib Khwaja, MD, FRCPath, University College London Hospitals NHS Foundation Trust, London, UK, discusses a retrospective analysis of real-world outcomes in patients with cold agglutinin disease (CAD), a type of autoimmune hemolytic anemia, highlighting common clonal characteristics and therapeutic strategies. This interview took place at the 67th ASH Annual Meeting and Exposition, held in Orlando, FL.
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Transcript
So cold agglutinin disease is kind of a part of the autoimmune hemolytic anemias, we traditionally think of warm and cold hemolytic anemia. And there’s been some changes over recent years with the WHO classification of cold agglutinin disease as a specific disorder itself and there’s a CAD-associated lymphoproliferative disorder as a histological definition...
So cold agglutinin disease is kind of a part of the autoimmune hemolytic anemias, we traditionally think of warm and cold hemolytic anemia. And there’s been some changes over recent years with the WHO classification of cold agglutinin disease as a specific disorder itself and there’s a CAD-associated lymphoproliferative disorder as a histological definition. And there are some lots of confusing terms, I think, in the world of CAD because you have secondary cold agglutinins that can be seen related to overt lymphoma, infection, and drugs and other such things. So we aim to look in our center at the outcomes of patients with cold agglutinin disease, their clonal characteristics, and some of the treatments that were given.
So we had a large group of over 40 patients who were treated in recent years. We found that the majority of those had a MYD88 clone, which is the typical clone that you see in CAD. There were a small proportion who were MYD88-positive, but they didn’t have an overt lymphoma, but they had this circulating IgM monoclonal protein. And with modern treatment outcomes, which are generally rituximab-based with combination chemotherapy, such as bendamustine and other agents, you can have really excellent outcomes. So you can have overall response rates of greater than 60-70%. We know that this is a chronic disorder, median survival in our cohort was over 14 years, which is really good, but there is morbidity associated with these patients. So those who have brisk hemolysis can develop thrombosis, you can have infections, you can have complications with this disorder. Certain therapies are not effective, we know about this from other sources, but we’ve confirmed it here. So steroids don’t typically have good responses and traditional things for warm hemolytic anemia such as MMF and those agents are not effective, which we’ve essentially confirmed in our findings. So I think it’s useful as a real-world data set and helpful to compare with other countries and other resources elsewhere.
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