Promoting Recognition, Diagnosis and Treatment of Cold Agglutinin Disease

By: Annenberg Center for Health Sciences
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  • Summary

  • Cold Agglutinin Disease is a rare, chronic hemolytic disorder, representing approximately 20% of all autoimmune hemolytic anemias. Catherine M. Broome, MD, and Alexander Röth, MD, provide an understanding of the pathogenesis of this disorder caused by cold-reacting IgM autoantibodies and reveal common clinical features. Because of the high mortality rate among those suffering from CAD, it is important to be aware of the diagnostic criteria, as well as the current and novel treatment options, with the goal of increasing hemoglobin levels, avoiding the need for transfusions, and improving circul
    Annenberg Center for Health Sciences
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Episodes
  • Pathogenesis and Clinical Features of Cold Agglutinin Disease

    Pathogenesis and Clinical Features of Cold Agglutinin Disease
    Aug 30 2021
    • Cold Agglutinin Disease (CAD, a type of Autoimmune Hemolytic Anemia, AIHA) is a rare disease, challenging at presentation and can present acutely or with more chronic symptoms
    • CAD is a subtype of AIHA (accounting for ~20% of all AIHA) caused by IgM autoantibodies, which tend to react at cold temperatures; Cold Agglutinin Syndrome (CAS) is also an AIHA mediated by IgM, associated with systemic disease, most commonly infection or malignancy
    • Hemolysis in CAD is complement-dependent with mainly extravascular hemolysis, typically in the liver
    • CAD affects ~one person per million every year; middle-aged and elderly people, 40–80 years of age with average age of onset 60 years of age; more common in women than men
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    19 mins
  • Interactive Case Scenario

    Interactive Case Scenario
    Aug 30 2021
    • Follow the case of a 62-year-old female who began to feel fatigue and shortness of breath in December 2010 through treatment to April 2017
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    6 mins
  • Emerging Treatments for Cold Agglutinin Disease

    Emerging Treatments for Cold Agglutinin Disease
    Aug 30 2021
    • Unmet medical need due to the high frequency of persistent anemia/hemolysis; immunochemotherapy is unsuccessful in at least 25% of cases because of treatment failure or toxicity; and small B-cell clone shows low proliferation activity and is difficult to target efficiently
    • Need for rapid acting therapy, especially in specific clinical settings, such as acute and severe exacerbations due to infections, major surgery, trauma, cardiac surgery
    • As of June 2021, there were six emerging treatments in trials, including Eculizumab, Bortezomib, Sutimlimab (BIVV009) in the CARDINAL and CADENZA trials, Pegcetacoplan (APL-2) and BIVV020 (both multiple and single IV doses)
    • The CADENCE Registry, a global patient registry launched in 2019, is an important tool in treating CAD and will provide perspective longitudinal data to advance understanding of patient demographics, clinical presentation and characteristics, co-morbidities and disease burden, patterns and use of CA
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    12 mins

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