Acute exacerbation of cold agglutinin disease during operation

provided the original work is properly cited. CC Cold agglutinin, an autoantibody that combines with the red blood cell (RBC) carbohydrate antigen, is known to trigger hemolysis with agglutination at temperatures of 0-4C [1]. Cold agglutinin combined with RBC in a peripheral body exposed to a cold environment returns to the center of the body and combines with the complement; these processes form the mechanism for chronic cold agglutinin disease. However, these processes can result in acute aggravation due to stimuli such as infection and trauma [2]. The authors experienced hemolysis during an operation in a patient with cold agglutinin disease although his temperature was maintained above 35.6C. A 67-year-old male patient diagnosed with bladder cancer was scheduled for a radical cystectomy with ileal conduit formation. He had been diagnosed with idiopathic cold agglutinin disease 20 years earlier. Hematologic conditions were stable without any medication. Hemoglobin was measured as 6.9 g/dl 2 days prior to the surgery. After 3 units of leukocyte-depleted RBC were transfused, the hemoglobin level increased to 9.9 g/dl. The cold agglutinin titer was 1 : 8192 and total bilirubin was 2.9 g/dl before the surgery. On the day of the surgery, the temperature of the operation room was raised to its maximum of 30C. An esophageal stethoscope with a temperature probe was inserted after induction to read the core body temperature (BT), and a skin temperature probe was attached to the forehead to read the peripheral BT. The body of the patient, excluding the site of surgery, was covered in cotton blankets, and 2 air warmers were used. A normothermic (37C) intravenous fluid administration set was used to elevate the temperature of all fluids. The inhalational gas supplied to the patient was set at 37C using a heated circuit. The core BT of the patient was 37C and his peripheral BT was 35.6C during the surgery. It was observed that the urine had a strong brown color [3], and at 60 minutes after anesthesia (30 minutes after the operation), blood tests showed hemoglobin at 11.0 g/dl, lactate dehydrogenase (LDH) at 494 IU/L, total bilirubin at 5.4 g/dl, and direct bilirubin at 2.1 g/dl. We decided that postoperative plasmapheresis was more appropriate than intraoperative plasmapheresis [4]. The estimated blood loss during the 600 minute surgery was 2800 ml, infused fluid HES 500 ml, and normal saline 4800 ml. Leukocyte-depleted RBC 6-units were transfused. Therapeutic plasmapheresis was executed for 180 minutes in the intensive care unit. The patient’s blood was extracted through an arterial line and reinfused through a central venous line. Both lines were passed through fluid warmers. Therapeutic plasmapheresis was completed without any complications. The test results were as shown in Table 1. The hemoglobin level was stable without any significant decrease; therefore, the patient was transferred to the general ward on postoperative day 5 (POD#5). During the intensive care unit (ICU) stay, the temperature taken from the arm pit was above 38C for the first 2 days and remained at 37-38C until the fifth day. After transfer to the general ward, RBC 1 unit was transfused on each of the following days: POD#8, POD#10, POD#13, and POD#15. The temperature taken from the arm pit remained at 38C. The patient was discharged on POD#16 with a hemoglobin level of 8.9 g/dl, and a month after the surgery (2 weeks after discharge), the hemoglobin measured at ambulatory care was 7.4 g/dl. There were no complications afterwards. In this case, although the cold agglutinin antibody number