Waldenstrom巨球蛋白血症合併高粘度症候群之積極性雙重過濾血漿分離療法

Intensive Double - Filtration Plasmapheresis for Hyperviscosity Syndrome in Waldenström's Macroglobulinemia

高粘度症候群為Waldenstrom 巨球蛋白血症常見之併發症，高粘度症候群治療主要以血漿交換或血漿分離療法為主。血漿交換雖成效不錯，唯輸液相關併發症仍是潛在之缺點；雙重過濾血漿分離術以半選擇性方式除去血中大分子而緩解症狀，唯過高之血中球蛋白常會造成血漿成份分離器之堵塞，影響療效。在此，我們報告一例 Waldenstrom 巨球蛋白血症合併之高粘度症候群採用雙重過濾血漿分離術療法。當遭遇分離器堵塞時，即刻更換分離器持續分離治療，一個療程之後，免疫球蛋白Ｍ的清除率高達73%，且視網膜出血，頭暈，頭痛及胸悶症狀緩解。治療過程中未有相關併發症發生。因此我們認為，積極性雙重過濾血漿分離術不但有血漿交換之療效，又可避免血漿交換之輸液併發症，對高粘度症候群應是一理想之治療方式。

Hyperviscosity syndrome is a common complication of Waldenstrom's macroglobulinemia. The treatment of choice is plasma exchange or plasmapheresis. Although the efficacy of plasma exchange has been well documented, transfusion-related complications remain as potential pitfalls. Double filtration plasmapheresis relieves the symptoms of hyperviscosity syndrome by semiselective withdrawal of macromolecules. However, occlusion of the filter system caused by the extraordinary amount of passing macromolecules limits the effectiveness of plasmapheresis. We report the use of double filtration plasmapheresis in treating with a patient with Waldenstrom's macroglobulinemia complicated with hyperviscosity syndrome. The filter was replaced each time when it became occluded. After one course of treatment, the immunoglobulin M concentration was reduced by 73% and the hyperviscosity-related features (retinal bleeding, headache, dizzness, and chest tightness) were relieved. No assoicated complications were noted. We conclude that intensive double filtration plasmapheresis appears to be an ideal choice for hyperviscosity syndrome in that it has efficacy comparable to that of plasma exchange but is devoid of transfusion-related complications.