Unusually Severe Course of Autoimmune Hemolytic Anemia / Cold Agglutinin Disease: A Case Report

Abstract

Abstract Introduction/Objective Autoimmune hemolytic anemia (AIHA) is a group of disorders with limited epidemiological and clinical data, characterized by hemolysis due to autoantibodies against red blood cell surface antigens. Cold agglutinins account for about 25% of all AIHA, which can agglutinate erythrocytes at 0-4 °C. Cold agglutinin disease (CAD) is a self limited disease and usually does not cause significant hemolysis. Here, we report a case with an unusually severe course for secondary CAD and role of plasma exchange in the management. Methods A 53-year-old male patient with no known past medical history presented to the emergency department with shortness of breath and syncope after a week of upper respiratory tract infection symptoms. The patient became profoundly anemic (presented with 8.1 g/dL hemoglobin level and progressively decline to 4.2 g/dL) in a short period of time. Upon initial evaluation, his peripheral blood smear demonstrated clumps of RBCs. Direct anti-globulin testing was negative for IgG but positive for C3 which is consistent with CAD. A respiratory PCR panel detected Rhinovirus. Mycoplasma and EBV IgM were negative. Additionally, chest X-ray showed right middle lobe pneumonia that was treated with antibiotics. Patient received multiple transfusions of pre-warmed pRBCS and showed initial improvement but eventually went into respiratory failure and cardiac arrest with return of spontaneous circulation after 8 minutes of CPR. Subsequently, plasma exchange was started. Only after intiation of plasma exchange, the patient’s ongoing hemolysis was stabilized. Conclusion This case report presents a patient with unexpectedly rapid and severe hemolysis from secondary cold agglutinin disease. Interestingly, the case appeared not to be caused by EBV or Mycoplasma pneumonia infection but Rhinovirus. Further studies confirmed patient had no autoimmune disorder or lymphoid malignancy that may had initiated secondary CAD. On the management aspect, pre-warmed RBC transfusions were not sufficient to stabilize the patient’s condition. Plasma exchange was able to control ongoing hemolysis within 2 sessions successfully.