地中海貧血患者的肝硬化是可避免的

經骨髓移植、鐵去除和抗HCV治療後,肝硬化明顯減輕

  2002年5月16 日─傳統觀點認為,地中海貧血患者的肝硬化是不可避免的。但5月7日出版的《內科學年報》中發表的一項回顧性研究發現,伴有肝硬化的地中海貧血患者經積極性治療後,令人驚奇的結果表示肝硬化的可治癒性。

  義大利佩扎羅Azienda Ospedale San Salvatore的Pietro Muretto醫師和同事寫道:「一些患者通過骨髓移植治癒了地中海貧血,經鐵去除治療後,肝硬化現象明顯減少。」

  491名地中海貧血患者經骨髓移植被治癒,其中6名患者經肝組織活檢被發現不完全或確定的肝硬化,在追蹤時間內肝硬化得到明顯的改善。

  這6名患者接受了鐵去除或抗病毒療法後,肝組織活檢發現,肝臟內的鐵儲備量顯著減少,其中4例活檢發現完全的鐵去除。所有患者的血清氨基轉移酵素水平均降低,5名恢復正常。所有患者的組織學炎症反應減輕,2名患者完全消失。追蹤活檢發現,所有患者不完全或確定的肝硬化減輕。

  作者寫道:「很明顯的,去除引起肝損傷的病因是治療肝臟纖維化和硬化的基礎。這篇報導應該能提醒患者和醫生,即使有什麼被認為是不可治癒的併發症,比如肝硬化,也應該堅持不懈地進行必要的治療『整合作用、抗病毒治療,以及最終的骨髓移植』。」

Cirrhosis Reversible in Thalassemia

After Bone Marrow Transplant, Iron Depletion, Anti-HCV Therapy Effective

By Laurie Barclay, MD
WebMD Medical News

Reviewed by Gary D. Vogin, MD

May 16, 2002 -- In a retrospective study described in the May 7 issue of the Annals of Internal Medicine, aggressive treatment of thalassemia in selected patients with cirrhosis, traditionally thought to be irreversible, surprisingly showed reversibility.

"In some patients in whom bone marrow transplantation has cured thalassemia, cirrhosis may be reversible after iron removal treatment," write Pietro Muretto, MD, and colleagues from the Azienda Ospedale San Salvatore di Pesaro in Italy.

Of 491 consecutive patients whose thalassemia was cured by bone marrow transplantation, 6 selected patients had incomplete or definite cirrhosis on liver biopsy and distinct improvement during follow-up.

After the patients received iron depletion and antiviral therapies, liver biopsies showed impressive reduction in liver iron stores, including 4 biopsies showing complete iron removal. Serum aminotransferase levels decreased in all patients and normalized in 5, and histologic evidence of inflammation decreased in all patients and disappeared in 2. Follow-up biopsies showed regression of incomplete or definite cirrhosis in all patients.

"Removing the cause of liver injury is clearly the cornerstone of treatment of fibrosis and cirrhosis," the authors write. "This report should alert patients and physicians to persist with necessary therapy (chelation; antiviral therapy; and, eventually, bone marrow transplantation), even if a complication thought to be irreversible, such as cirrhosis, occurs."

© 2002 WebMD Inc. All rights reserved.

    
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