台中69歲黃女士十多年來因頭暈問題困擾不已,曾經至台中各大醫院耳鼻喉科、神經內科和心臟科求診,始終未能找到頭暈的根本原因。黃女士最近到仁愛長庚合作聯盟醫院耳鼻喉科張耕閤醫師門診就診,張醫師詳細詢問黃女士病史後,發現她長期流鼻血,並在舌頭尖端與下唇發現一些類似血管瘤的小突起,進一步使用鼻腔內視鏡檢查,發現其鼻腔粘膜上散布著許多小血管瘤,並有出血現象。此外,張醫師也注意到黃女士的眼白非常白,走進診間時明顯無力,且呼吸急促。
張醫師懷疑黃女士可能患有遺傳性出血性血管增生症(Hereditary Hemorrhagic Telangiectasia, HHT,又名Osler-Weber-Rendu病),立刻為她抽血檢查血色素,結果報告顯示黃女士的血色素值極低,只有3.6(女性正常值範圍為11.5-16.5),已達到危險水平。張醫師立刻聯絡黃女士要她到該院急診接受治療,在急診接受輸血治療後,暈眩等症狀明顯改善。黃女士回診時告訴張醫師,感覺宛如重獲新生,非常感謝張醫師終於為她找出病因,不再飽受暈眩之苦。
張醫師表示,遺傳性出血性血管增生症是一種罕見的遺傳性疾病,盛行率大致在每5000-10,000人中有1人受到影響。該病會造成慢性鼻腔出血,血色素持續降低,身體代償,習慣了,但會造成長期頭暈、無力、喘等症狀。尤其是當患者同時存在鼻中隔彎曲、鼻過敏、或鼻竇炎時,由於鼻腔常有阻塞與流鼻涕,擤鼻涕時更容易出血。治療上,患者需要先檢查是否有合併大腦、肺部、肝臟的大型血管瘤。鼻腔出血點通常需要使用內視鏡手術方式,將粘膜上的血管瘤去除,可以用雷射或者無線射頻消融的方式進行。
張醫師說,治療方法多樣,包括:荷爾蒙相關藥物如他莫昔芬和瑞洛昔芬、抗血管生成藥物如貝伐單抗(Avastin)、抗纖溶藥物如Tranexamic acid(Lysteda)、皮膚移植以及激光或高頻電流的消融療法等。病患可能需要結合藥物治療和手術以獲得最佳效果。另外,調整生活方式,如避免某些藥物和食物,保持鼻腔濕潤和潤滑,也是減少HHT鼻出血風險的重要一環。
張醫師指出,患者的生存率和死亡風險也受到這種疾病的影響。研究發現,HHT患者的生存率較低,死亡風險隨著年齡的增加而增加,與一般人相比,HHT患者的中位死亡年齡也較低。因此,對於長期存在頭暈、無力和流鼻血等症狀的患者,及早到門診接受檢查和治療是非常重要的。
張醫師強調,持續不明原因頭暈和流鼻血可能是罕見疾病的警訊,若民眾長期有類似症狀,建議應及時到醫院進行檢查,以確定病因並接受適當治療,以提高生活品質並降低潛在的健康風險。
仁愛長庚合作聯盟醫院耳鼻喉科張耕閤醫師
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