A retrospective analysis hints at the wide range of cold agglutinin disease (CAD) clinical behavior. Koudouna et al. investigated the characteristics of 8 patients with CAD at the time of diagnosis . Median age was 62 years, and 5 patients were women. Hematologic malignancies constituted 50 % of underlying medical conditions; in 37 %, hepatitis B/C was the associated disease, and in 13 %, autoimmune disorders. The median hemoglobin level at presentation was 8.9 g/dL. Slightly elevated serum CRP and ferritin levels represented common findings. All patients had cold-sensitive antibodies, with one also showing cryoglobulins. Cold agglutinins titers were disproportionate to the degree of hemolysis in 4 patients with underlying lymphoproliferative disorders. Low serum monoclonal IgM prevailed in 4 cases even in the presence of hypogammaglobulinemia. Fatigue dominated among symptoms, followed by hepatomegaly and jaundice, skin complications, and splenomegaly. The patient who had cryoglobulinemia showed skin necrosis due to vascular occlusion.
All patients initially received corticosteroids. Complete or partial responses were observed in 3 patients with primary CAD treated with corticosteroids, splenectomy and mycophenolate mofetil. Additional approaches included plasma exchange and anti-CD20 therapy. In 5 cases, additional treatment for the underlying disease was administered. During a median follow-up of 42.5 months, 2 patients died from infection and sepsis, while another 2 completely recovered and the remaining patients experienced relapses and remissions.