Updated: 1/22/2019

Cold-Agglutinin Disease

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Snapshot
  • A 21-year-old female presents with pain in her fingers and toes and has noticed that they turn purple. Other complaints include a continuous, nonproductive cough with low grade fever. She believes that her symptoms are due to the winter temperature, as her symptoms improve when she is indoors. On physical exam, there is a bluish discoloration of the ears, nose-tip, and fingers. Pulmonary auscultation is notable for rales.
Introduction

  • Immunohemolytic anemia at low temperatures
    • IgM antibodies directed against red blood cells
      • low temperatures increases antibody avidity to its antigen
      • can lead to complement fixation
    • extrinsic hemolytic anemia
  • Cold agglutinin antibody production can be secondary to:
    • Mycoplasma pneumoniae
    • mononucleosis (EBV and CMV)
    • HIV
    • B-cell neoplasms
      • Waldenstrom macroglobulinemia
    • idiopathic
Presentation
  • Symptoms
    • anemia
      • pallor, dyspnea
    • acrocyanosis and levido reticularis
      • can appear when exposed to cold temperatures
        • acrocyanosis
          • purplish discoloration of the tip of the nose, ears, fingers, and toes
      • can improve when warm
        • insignificant reactive hyperemia
          • differentiates it from Raynaud phenomenom
Evaluation
  • Direct Coombs test
    • positive for anti-C3
    • negative for anti-IgG
  • Elevated cold agglutinin titers
  • Low C3 and C4 levels
    • secondary to consumption
  • Red blood cell aggregation on peripheral blood smear
Treatment
  • Dependent on disease severity
    • avoiding the cold
    • rituximab
      • in those with severe hemolysis
      • may be given with fludarabine
    • other options can be used as well
      • glucocorticoids, cyclophosphamide, cyclosporine, IVIg

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