川崎病に対する血漿交換療法の実際(<特集>血管炎症候群とアフェレシス)

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  • Plasma Exchange for Patients with Kawasaki Disease

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Intravenous immunoglobulin (IVIG) has been improved the outcome of patients with Kawasaki disease. With its favorable survival, the treatment for Kawasaki disease becomes focused on prevention and treatment of the severe late complications such as the coronary arterial dilatation or aneurysm. The efficacy of the treatment of infliximab or apheresis for Kawasaki disease patients who fail to respond to initial IVIG therapy has begun to be accepted recently. However, there are technical difficulties due to the small amount of blood volume in performing apheresis in children such as the negative effect on the hemodynamics, the difficulty in insertion of vascular accesses, and the hypothermic effect. We have developed a way to perform apheresis in hemodynamically unstable child patients such as neonates and so applied apheresis to Kawasaki disease as well, using a dedicated circuit, small catheter, and warmer coil. In addition, a pre-dialyzation technique before performing apheresis using a mixture of packed red blood cells and frozen flesh plasma enables apheresis to begin without dropping in the blood pressure. We have performed apheresis on 12 patients with Kawasaki disease, including a hemodynamically unstable child who needed assisted circulation, and had favorable outcomes. Apheresis for IVIG-resistant Kawasaki disease is effective for preventing coronary artery complications.

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