5)自己免疫疾患における小児のアフェレシスの特殊性(〈シンポジウム〉各科領域におけるアフェレシス療法)

概要

論文の詳細を見る
Plasmapheresis and plasma-exchange (PP/PE) are applied to a various kind of pediatric autoimmune diseases. There is no established indication for therapeutic apheresis, but diseases that are fulminant and refractory to conventional therapies seem to be the most common indication. Recently, we performed PP/PE in seven patients with rheumatic diseases. They consisted of systemic arthritis (3), lupus nephritis (2), large vessel arteritis (1), and autoimmune lymphoproliferative syndrome (1). The patients varied in age from 10 months to 15 years. In younger patients, there existed some important issues. Firstly, adequate vascular access to provide enough blood-flow (>20ml/min) was required. Secondly, minimization of a priming volume was necessary because of a low blood volume in childhood. This was achieved by a use of special circuit with a low volume and priming of lines with packed red blood cells or FFP. PP/PE was effective in five of seven patients, but the effectiveness was transient in two patients. Adequate immunosuppressive therapies in combination with PP/PE are needed to maintain the effect of PP/PE.