Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Thirty-six patients (40%) received transfusions during their disease course, and 82% received drug therapy. An underlying hematologic disorder was detected in 69 patients (78%). The most common symptom was acrocyanosis (n = 39 ), and many had symptoms triggered by cold (n = 35 ) or other factors (n = 20 ). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. Median age at symptom onset was 65 years (range, 41 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 91 years). Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia.
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