嚴重複合型免疫缺乏症(英語:Severe combined immunodeficiency,縮寫 SCID),泛指一群罕見的先天遺傳性疾病,患者因為免疫功能缺乏 (例如 B 細胞或 T 細胞的發育不全、缺陷),使其幾乎或完全無法對病原體具有免疫力,導致嚴重的重複性感染,來源包括細菌、病毒及黴菌,多數患者在出生後一年內死亡。[1]
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發生率
疾病分類
可導致 SCID 的基因非常多樣,目前已經被證實的基因數量已經超過 20 個。[2]
名稱 | 致病基因 | 淋巴球有無 | 遺傳模式 | ||
---|---|---|---|---|---|
T 細胞 | B 細胞 | NK 細胞 | |||
X-linked SCID | γ chain for IL2,4,7,9,15,21 | - | +
(無功能) |
- | X-linked |
JAK3-SCID | JAK3 | - | +
(無功能) |
- | AR |
IL7Ra-SCID | IL7RA | - | + | + | AR |
CD45 deficiency | CD45 | - | + | - | AR |
ADA deficiency | ADA | - | - | - | AR |
RAG-deficient SCID | RAG1 | - | - | + | AR |
RAG2 | - | - | + | AR | |
Immunodeficiency 17 | CD3 | - | + | + | AR |
ZAP-70 kinase | ZAP70 | 僅有 CD4+ | + | + | AR |
MHC II deficiency | MHC class II | 僅有 CD8+ | + | + | AR |
參考文獻
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