Clinical and laboratory characteristics of patients with cold agglutinin disease: A retrospective analysis at a tertiary medical center

Background: Cold agglutinin disease (CAD) is relatively rare and has primarily been reported as retrospective case series.

Aim: We reviewed our experience with CAD to shed light on this disease.

Study settings and design: This was a retrospective review of all patients with CAD managed at our institution between 2007 and 2018.

Materials and methods: The study was approved by our institutional review board. We extracted patients' demographic, clinical, and laboratory data, blood transfusions, and outcomes from their electronic medical records.

Statistical analysis used: Statistical analysis was performed using SPSS version 17. The method of Kaplan-Meier was used to plot survival curves.

Results: Forty-eight patients fulfilled the inclusion criteria for CAD. The median age of patients was 73.1 (range, 43-99) years; 36 (75%) were female. The majority (n = 38; 79.2%) of patients were Caucasians. Most patients (n = 25, 52.1%) presented with symptomatic anemia. Eight patients were asymptomatic. The median hemoglobin level was 8.6 g/dL (range, 3-12 g/dL); 7 (14.6%) patients had concurrent thrombocytopenia. Lactate dehydrogenase was elevated in 40/47 (85.1%) patients and haptoglobin was below normal in 35/46 (76.1%) patients. Coagulopathy was observed in 19 (52.8%) of 36 patients. Sixteen (33.3%) patients required blood transfusion during admission at the time of diagnosis with a median number of 3.5 red blood cell units. Twenty-five (52.1%) patients were alive after a median follow-up of 50.1 months. The 5-year and 10-year survival was estimated at 58.2% and 30.8%, respectively.

Conclusion: CAD poses considerable burden on patients and health-care systems. Patients vary widely in their disease severity and course.