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  • Pathogenesis and Clinical Features of Cold Agglutinin Disease

    Pathogenesis and Clinical Features of Cold Agglutinin Disease
    2021/08/30
    • 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 分
  • Interactive Case Scenario

    Interactive Case Scenario
    2021/08/30
    • 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 分
  • Emerging Treatments for Cold Agglutinin Disease

    Emerging Treatments for Cold Agglutinin Disease
    2021/08/30
    • 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 分
  • Current Treatment Approaches for Patients with Cold Agglutinin Disease

    Current Treatment Approaches for Patients with Cold Agglutinin Disease
    2021/08/30
    • Treatment goals should be to improve anemia by increasing Hb levels, avoid transfusion, improve circulatory symptoms caused by cold temperatures and reduce inflammatory symptoms
    • There are a number of non-pharma treatment options, including thermal protection and cold avoidance and transfusions (avoid cold transfusions)
    • Corticosteroids should not be used to treat CAD
    • There are several first-line and second-line pharmaceutical treatment strategies, including Rituximab monotherapy, Rituximab-Bendamustine and Rituximab-Fludarabine combination therapies and Bortezomib monotherapy
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    8 分
  • Diagnosing Cold Agglutinin Disease

    Diagnosing Cold Agglutinin Disease
    2021/08/30
    • To identify potential patients, a diagnostic workup of AIHA is essential, including antibody type, patient history and physical exam, hemoglobin levels and blood cell counts, blood smear examination and direct antiglobulin test (DAT)
    • Diagnostic criteria for CAD include chronic hemolysis, polyspecific DAT positive, monospecific DAT strongly positive for C3d, CA titer ≥64 at 4°C and a bone marrow evaluation (advised in patients with CAD at diagnosis)
    • Hemoglobin is the most direct indicator of clinical severity
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    7 分