https://orcid.org/0000-0003-1202-8919
Abstract
Evans syndrome is a rare haematological disease that may cause several complications during heart surgery. Herein we documented heart valve surgery in a patient with Evans syndrome who was receiving monoclonal antibody therapy, and valve replacement was successfully performed via prophylactic measures against haemolysis.
INTRODUCTION
Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune haemolytic anaemia and immune thrombocytopaenia and/or immune neutropenia [1]. The combination of autoimmune haemolytic anaemia and immune thrombocytopaenia can cause intravascular haemolysis and a fatal bleeding tendency during the perioperative period of cardiac surgery [2]. Although few reports exist on surgery for patients with ES, to the best of our knowledge, this is the first report on a patient with ES who successfully underwent double valve replacement after rituximab administration.
CASE REPORT
A 49-year-old man was admitted to our hospital due to severe general malaise and dyspnoea. Clinical examinations suggested congestive heart failure due to rheumatic heart valve disease, aortic valve stenosis and mitral valve stenosis. Complete blood count confirmed anaemia and thrombocytopaenia (haemoglobin, 6.3 g/dl; platelet count, 3.8 × 104/dl) at admission; reticulocyte count had increased by 27.3%. Lactate dehydrogenase was increased to 738 IU/l. Direct and indirect Coombs tests and direct antiglobulin tests were positive. Platelet-associated immunoglobulin G was increased to 548 ng/107 cells. The haematologist excluded other causes of acquired immune cytopenia such as systemic lupus erythematosus. He was ultimately diagnosed with ES.
Prednisolone administration (75 mg/day) was initiated on hospitalization day 5. Response to steroid treatment was not good; thus, he was administered 4 doses of the monoclonal antibody rituximab (375 mg/m2 weekly). Consequently, anaemia and thrombocytopaenia improved, and prednisolone was gradually tapered (20 mg/day) 2 months after rituximab administration. Haemoglobin and platelet concentrations remained at ∼10 g/dl and 13 × 104/dl, respectively. He was subsequently referred to us for a cardiac operation. Echocardiography revealed 0.9 cm2 of the aortic valve area and 1.1 cm2 of the mitral valve area. The lower left ventricular function was observed (ejection fraction: 45.7%).
He underwent double valve replacement 3 months after rituximab administration. Methylprednisolone (100 mg) was intravenously administered at the introduction of anaesthesia. The standard cardiopulmonary bypass (CPB) was performed using a centrifugal pump (Revolution, LivaNova, London, UK) and heparin-coated circuits. Both the valves were replaced with a 21-mm Carpentier-Edwards pericardial valve (Edwards Lifesciences, Irvine, CA, USA) for the aortic and a 29-mm Carpentier-Edwards pericardial valve for the mitral valve. Thromboelastometry (ROTEM Delta; TEM International, Munich, Germany) was used for intraoperative haemostasis testing. Red blood cell and platelet transfusions were necessary at the end of surgery. The surgery was completed without any difficulty or bleeding tendency. Surgery time was 6 h 37 min and CPB time was 195 min. Blood loss was 177 ml. The intubation time was 11 h; 40-mg prednisolone was administered 2 days postoperatively, and then returned to 20 mg/day. The postoperative course was uneventful, and haemolytic anaemia did not occur during hospitalization (Fig. 1). The patient was discharged on postoperative day 17. The institutional review board approved this study; the patient provided informed consent.
Time course during the perioperative period. C-Plt: concentrated platelets; CRC: concentrated red cells; Hb: haemoglobin; LDH: lactate dehydrogenase; Plt: platelet cell; POD: postoperative day.
DISCUSSION
Special care is required in perioperative management of patients with haematological disorders, especially ES. It is important to remit haematological disorders preoperatively. The present patient had recently been diagnosed with ES; high-dose steroid therapy had been initiated. Given his poor response to steroid treatment, he was administered rituximab. Although immunosuppressants are second-line therapy [3], they were not preferred preoperatively. Rituximab is a chimeric monoclonal antibody against the CD20 protein and is thought to selectively damage B-lymphocytes and suppress antibody production. The efficacy rate of rituximab is 87%, there are fewer side effects and the duration is ∼12 or more months [3]. The patient underwent surgery while the effect of rituximab remained. No ES exacerbation was observed, but if ES relapsed, intravenous immunoglobulin, immunosuppressants and increased steroid doses were planned. Bioprosthetic valves were inserted in the young patient because anticoagulant therapy became difficult when ES relapsed [4], and steroids would be administered for a lifetime. The reticuloendothelial system is involved in the extravascular haemolytic reaction and complement activity is minor in ES [5]. To prevent haemolytic anaemia, we used the centrifugal pump that provides a lesser degree of haemolysis than a roller pump, and heparin-coated circuits were used to suppress platelet consumption during CPB. The patient was followed up by the haematological section of our hospital, and no ES relapse and heart failure were noted 1 year postoperatively.
In summary, we performed successful double valve replacement in a patient with ES after rituximab administration. With proper management, ES does not cause complications during heart surgery.
ACKNOWLEDGEMENTS
The authors thank Keiichi Ono for assistance with clinical data acquisition for haematological disorder. They also thank Enago (www.enago.jp) for English language review.
Conflict of interest: none declared.
