巨噬細胞活化症候群與自體免疫疾病

Macrophage Activation Syndrome and Autoimmune Disease

林泓科(Hung-Ke Lin)

《內科學誌》 25卷3期 (2014/06) Pp. 195-198

https://doi.org/10.6314/JIMT.2014.25(3).09

摘要

從1985年Hadchouel M.提出巨噬細胞活化症候群，這個疾病被歸類為次發型噬血球性淋巴組織球增生症，相關的自體免疫疾病以系統性幼年型關節炎為最常見。其病理機轉為自然殺手及T細胞的免疫缺損，不足以清除和壓制過度活化的免疫細胞，出現了免疫激化現象，影響全身器官，表現出發燒，出血傾向，血球低下，和中樞神經症狀等。目前的診斷雖沿用噬血球性淋巴組織球增生症－2004歸類準則，但修正診斷標準的提議未曾間斷；治療仍以類固醇為一線藥物，來控制過盛的免疫系統，而其他的二線藥物雖多，但仍有待共識。隨著分子生物研究，新的診斷方式和治療藥物有著長足的進步，有賴臨床醫師對各種早期徵候的警覺性，早期診斷，早期治療，方能有好的預後。

關鍵字

巨噬細胞活化症候群（Macrophage activation syndrome） ； 噬血球性淋巴組織球增生症（Hemophagocytic lymphohistiocytosis） ； 自體免疫疾病（Autoimmune disease） ； 免疫過度活化（Immune over activation） ； 血球吞噬現象（Hemophagocytosis）

並列摘要

Macrophage activation syndrome which was first described by Dr. Hadchouel M. in 1985 by clinical observation has been classified as secondary form of hemophagocytic lymphohistiocytosis (HLH). The most common associated autoimmune disease is juvenile idiopathic arthritis. The pathogenesis is that the impaired natural killer and cytotoxic T cell have problem in suppressing and clearing the reactivated immune cell. The overacting immune system affects the whole body and presents fever, bleeding tendency, pancytopenia, central nervous system symptoms and so on. The adjustment of diagnostic criteria has been proposed since the HLH-2004 diagnostic criterion is used as diagnostic tool. Steroid remains the first choice of treatment, but there is still no conclusion as for the secondary line drugs. Although there is much progress in recent years, early diagnosis and well treatment still rely on highly suspicious on recognition of the early presentation.