Ann Thorac Surg 2001;71:351-352
© 2001 The Society of Thoracic Surgeons
Case report
Cardiac operation for a patient with autoimmune hemolytic anemia with warm-reactive antibodies
Masahiko Onoe, MD, PhDa,
Tatsuo Magara, MD, PhDa,
Yoshio Yamamoto, MDa
a Department of Cardiovascular Surgery, Shiga Seijinbyo Medical Center, Shiga, Japan
Accepted for publication March 22, 2000.
Address reprint requests to Dr Onoe, Department of Cardiovascular Surgery, Kinki University School of Medicine, 377-2 Ohno-higashi, Osaka-sayama, Osaka 589-0014, Japan
e-mail: smiling_onoe{at}hotmail.com
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Abstract
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We present a case of a patient with autoimmune hemolytic anemia with warm-reactive antibodies who underwent successful coronary artery bypass grafting. We discuss some of the important problematic issues related to perioperative management.
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Introduction
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Autoimmune hemolytic anemia (AIHA) with warm-reactive antibodies is a rare type of acquired hemolytic anemia caused by an antibody reaction against a patients own erythrocytes at normal body temperature. Several studies have reported the use of cardiac operation for patients with various forms of hemolytic anemia, such as sickle cell anemia, cold hemoagglutinemia, and hereditary spherocytosis [15]. We describe a patient with AIHA with warm-reactive antibodies who underwent successful coronary arterial bypass grafting (CABG) without homologous blood transfusion.
A 58-year-old man was admitted to our hospital because of severe general malaise, palpitation, and dyspnea. Blood analysis revealed severe anemia with a hematocrit value of 19.7% and reticulocyte count of 685%. Cytologic study showed spherocytosis and hemagglutination at 37°C. Urinary analysis demonstrated only a slight occult blood reaction and no occult blood was detected in the patients stool. The total plasma bilirubin level was also observed to have increased to 5.3 mg/dL and lactate dehydrogenase to 1,937 IU/L. Direct and indirect Combs tests were both positive and no cold agglutinin was detected. Therefore, the patient was diagnosed as having AIHA with warm-reactive antibodies.
Electrocardiography on admission showed ST depression at V4 to V6, suggesting ischemic heart disease. Coronary angiography revealed 90% stenosis at the ostium of the left coronary artery indicating CABG.
The patient was given prednisolone at 70 mg/day beginning on the 3rd day of admission, with the dose tapered to 35 mg/day on the day before operation. In response to this therapy, anemia gradually improved. After improvement of anemia, autologous blood donation of 1,200 mL was performed.
The patient underwent CABG 74 days after admission, when the hematocrit value increased to 40%. The patient was placed in a supine position and the heart was exposed through a midline sternotomy. At the same time, the greater saphenous vein was harvested from the left lower leg for vein grafts. Then cardiopulmonary bypass was established by inserting an arterial perfusion cannula into the ascending aorta and a straight venous return cannula into the right atrium through the right atrial appendage. An ordinary roller pump (Model 7400, Sarns, Inc, Ann Arbor, MI) and hollow fiber membrane oxygenator (LH 760, JMS, Hiroshima, Japan) were used for cardiopulmonary bypass. The ascending aorta was cross-clamped and the heart was arrested by infusion of Youngs solution, which consisted of crystalloid cardioplegia with high-dose magnesium. Then St. Thomas cardioplegic solution at 4°C was infused antegradely through a small infusion cannula placed in the ascending aorta. Under conditions of mild hypothermia (blood temperature 30°C), two saphenous vein grafts were anastomosed to the left anterior descending artery and left circumflex artery. Perioperatively, autologous donated blood was transfused, and no homologous blood was used. The hematocrit value was 28% immediately after the surgical procedure and 33.8% the following day.
For the first 2 postoperative days methylprednisolone (100 mg/day) was administered intravenously. Then oral prednisolone (30 mg/day) was begun on the 3rd postoperative day. The dosage of prednisolone was maintained until the patient was discharged. The postoperative course was uneventful and no recurrence of hemolytic anemia was observed. The patient was discharged 67 days postoperatively, when the hematocrit value was 36.1%.
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Comment
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Autoimmune hemolytic anemia is caused by autoantibodies produced against antigens on the surface of erythrocyte membranes, resulting in severe anemia due to hemolysis after antigen-antibody reaction [6]. In general, AIHA is classified as follows based on the behavioral characteristics of the antibodies involved: warm antibody types, including AIHA with warm-reactive antibodies, and cold antibody types, including cold agglutinin disease (CAD) and paroxysmal cold hemoglobinuria (PCH).
Cardiopulmonary bypass during cardiac operations reportedly injures and weakens the erythrocyte membrane, resulting in a reduced erythrocyte life span [7]. Therefore, perioperative hemolysis may represent a serious problem in cardiac operations in AIHA patients. Several studies have described cardiac operations in patients with congenital hemolytic anemia, including hereditary spherocytosis and sickle cell disease [15]. Moreover, one study described the influence of cardiac operations and extracorporeal circulation in patients with CAD [8]. However, our report describes a successful cardiac operation in a patient with AIHA with warm-reactive antibodies. For cardiac procedures in such AIHA patients, the following important considerations have been raised: (1) the possibility of inducing severe hemolysis due to extracorporeal circulation despite achieving remission of anemia by steroid hormone therapy, (2) the risk of side effects induced by high-dose steroid hormone therapy, and (3) the possibility of inducing hemolysis by perioperative homologous blood transfusion. In previous cardiac operation studies in patients with hereditary hemolytic anemia, to prevent hemolysis due to fragility of erythrocyte membranes the oncotic pressure was maintained by circulating the priming solution supplemented with plasma during cardiopulmonary bypass [3] or by using a centrifugal pump along with administration of antihemolytic agents [4]. In addition, preoperative splenectomy has been recommended [5]. In patients with AIHA, there is no fundamental problem with the erythrocyte membranes themselves. In the spleen, however, erythrocytes bound to antierythrocyte autoantibodies (IgG) are phagocytosed by macrophages after recognition through IgG-Fc receptors, mainly resulting in anemia in AIHA patients [9]. Therefore, we used a conventional roller pump during cardiopulmonary bypass beginning in the preoperative stage when antibody production was sufficiently inhibited. In terms of high-dose steroid hormone therapy, the initial dose of 70 mg/day was tapered to 35 mg/day before operation and steroid administration was resumed immediately after operation to prevent steroid deficiency or the rebound phenomenon. During the perioperative stage, a sufficient dose of antibiotics and antifungal agents was administered to prevent infection. Moreover, to avoid homologous blood transfusion, 800 mL of autologous blood was stored preoperatively, and 400 mL of autologous blood was also stored on the day of operation in the operating room.
The patient, after undergoing the above method, showed a satisfactory postoperative course almost identical to that of patients who undergo conventional cardiac operations. Moreover, no signs of abnormal perioperative hemolysis were observed in this patient. Therefore, we conclude that even in patients with AIHA with warm-reactive antibodies, when remission of anemia is achieved by sufficient preoperative therapy, conventional cardiopulmonary bypass, surgical procedures, and perioperative management are sufficient.
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