Interferon Therapy in an Infant with Kasabach-Merritt Syndrome

幼兒Kasabach-Merritt症候群使用幹擾素(Intron-A)之治療經驗

葉國偉(Kuo-Wei Yeh) ； 江東和(Tang-Her Jiang) ； 洪悠紀(Iou-Jih Hung)

《Acta Paediatrica Sinica》 36卷5期 (1995/10) Pp. 368-372

https://doi.org/10.7097/APS.199510.0368

摘要

Kasabch-Merritt症候群爲一罕見，但確能致命的疾病，多好發於嬰幼兒。在臨床上表現出巨大血管瘤併凝血機能異常。傳統上多以類固醇爲主要治療方式，邇來幹擾素的使用亦有不錯的治療效果。在本病例的治療經驗上，我們發現使用較低劑量的幹擾素，在短期療程後也有令人滿意的結果。
病人爲－38天大的幼兒，出生後便發現右腹側有一暗紅色硬塊且持續擴大，經核磁共振攝影檢查疑似爲一巨大血管瘤。住院時亦發現有凝血異常。初時接受一般輸血治療併使用類固醇，但臨床症狀及實驗檢查數據顯示病情惡化。在76天大時開始使用幹擾素(Intron-A)治療。於使用後14天，發現纖維蛋白原開始上昇並維持在100mg/dl以上。使用後21天，發現臨床上血管瘤變得較小較軟，症狀有明顯改善。使用後80天，血小板恢復正常。幹擾素使用122天後停藥。追蹤病人的情形，並無惡化的現象。

關鍵字

Kasabach-Merritt症候群 ； 幹擾素 ； 血管瘤

並列摘要

An infant with Kasabach-Merritt syndrome was treated with interferon-alfa 2b. Clinical and hematological responses were followed. lntron-A (Schering-Plough) was administered to a two and half-month- old male infant after failure of most other conventional therapies. A daily dose of 100,000u/Kg was given subcutaneously for a total of 122 days. Clinical and laboratory aggravation was observed in the first eight days. The fibrinogen level increased to ＞100mg/dl after two weeks of Intron-A treatment. Local inflammatory signs began to improve after 21 days. The platelet count became normal after 80 days of treatment. During follow-up, there was continuous regression of the tumor.

並列關鍵字

Kasabach-Merritt syndrome ； infant with giant hemangioma ； treatment with interferon alfa-2b