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Intervention Review

Blood transfusion for preventing stroke in people with sickle cell disease

  1. Ceri Hirst1,*,
  2. Winfred C Wang2

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 21 JAN 2002

Assessed as up-to-date: 11 JUN 2009

DOI: 10.1002/14651858.CD003146

How to Cite

Hirst C, Wang WC. Blood transfusion for preventing stroke in people with sickle cell disease. Cochrane Database of Systematic Reviews 2002, Issue 1. Art. No.: CD003146. DOI: 10.1002/14651858.CD003146.

Author Information

  1. 1

    AstraZeneca, Alderley Park, Cheshire, UK

  2. 2

    St Jude Children's Research Hospital, Department of Hematology/Oncology, Memphis, Tennessee 38105, USA

*Ceri Hirst, AstraZeneca, Parklands (90HW2Q1), Alderley Park, Cheshire, SK10 4TG, UK. Ceri.Hirst@astrazeneca.com. criddington@hotmail.com.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 21 JAN 2002

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This is not the most recent version of the article. View current version (14 NOV 2013)

 

Abstract

  1. Top of page
  2. Abstract
  3. Plain language summary
  4. 摘要

Background

In sickle cell disease (SCD), a common inherited haemoglobin disorder, abnormal haemoglobin distorts red blood cells, causing anaemia, vaso-occlusion and dysfunction in most body organs. Stroke affects around 10% of children with sickle cell anaemia and recurrence is likely. Chronic blood transfusion dilutes the sickled red blood cells, reducing the risk of vaso-occlusion and stroke. However, side effects can be severe.

Objectives

To assess risks and benefits of chronic blood transfusion regimens in people with SCD to prevent first stroke or recurrences.

Search methods

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings.

Last search of the Group's Trials Register: 22 May 2009.

Selection criteria

Randomised and quasi-randomised controlled trials comparing blood transfusion as prophylaxis for stroke in people with SCD to alternative or no treatment.

Data collection and analysis

Both authors independently assessed trial quality and extracted data.

Main results

Searches identified two eligible trials. One compared a chronic transfusion regimen for maintaining sickle haemoglobin lower than 30% with standard care in 130 children with SCD judged (through transcranial doppler ultrasonography) as high-risk for first stroke. During the trial, 11 children in the standard care group suffered a stroke compared to one in the transfusion group. This 92% relative risk reduction meant the trial was terminated early. Thirty months treatment was planned, but median follow up was 21.1 months. The transfusion group had a high complications rate: iron overload; alloimmunisation; and transfusion reactions. The second trial investigated risk of stroke when transfusion was stopped after at least 30 months. The trial closed early due to a significant difference in risk of stroke between participants who stopped transfusion and those who continued (for whom it was deemed unsafe to recommend discontinuation), as measured by abnormal velocities on Doppler examinations, OR 0.02 (95% CI 0.00 to 0.43). No trials were identified investigating transfusion for preventing recurrence of stroke.

Authors' conclusions

These trials demonstrated a significantly reduced risk of stroke in participants receiving regular blood transfusions. Data from a follow-up trial indicate individuals may revert to former risk status if transfusion is discontinued. Degree of risk must be balanced against the burden of chronic transfusions. Further research is required examining the use of transfusion in preventing secondary stroke, and further defining risk factors for stroke, to avoid unnecessarily starting children on blood transfusions.

 

Plain language summary

  1. Top of page
  2. Abstract
  3. Plain language summary
  4. 摘要

Regular blood transfusions to prevent a stroke in people with sickle cell disease

In sickle cell disease there are fewer red blood cells. This means less oxygen is carried to tissues and there are problems throughout the body. Sickled red blood cells can block blood vessels in the brain, leading to strokes. We aimed to compare long-term blood transfusion schedules to other transfusion schedules or other ways of preventing stroke. There are two trials in the review. The results showed that regular blood transfusions reduce the risk of stroke significantly by diluting sickled cells. There are important adverse effects (iron overload, infection transmitted through blood and reactions to the transfusions). When the treatment stopped, people returned to having a high risk of stroke. This may be the same level of risk as at the start of transfusion. When using this treatment the burden of long-term transfusion should be weighed against the degree of risk of stroke. Further research should look at using transfusion to prevent secondary stroke and defining further risk factors for stroke. This would avoid starting children on blood transfusions if these are not needed.

 

摘要

  1. Top of page
  2. Abstract
  3. Plain language summary
  4. 摘要

背景

輸血預防鐮刀型貧血症(sickle cell disease; SCD)患者中風

鐮刀型貧血症是一種常見的遺傳性血紅素疾病,異常的血紅素堆積在紅血球,造成貧血、血管阻塞(vasoocclusion)及許多身體器官的異常。中風約影響10%的鐮刀型血球貧血症病童,且有可能復發。長期輸血可稀釋鐮刀型紅血球,降低血管阻塞和中風的機會。然而,其副作用卻可能很嚴重。

目標

評估鐮刀型貧血症接受慢性輸血療法,預防初次中風或復發的風險與益處。

搜尋策略

我們搜尋了Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register資料庫,其主要是由廣泛搜尋電子資料庫所找到的參考書目,以及手動搜尋相關期刊及會議摘要冊所組成。最近搜尋Group's Register的時間:2006年11月。

選擇標準

比較鐮刀型貧血症患者,採用輸血治療與其他替代療法或無治療以預防中風的隨機與半隨機對照試驗(Randomised and quasirandomised controlled trials)。

資料收集與分析

兩位作者都獨立評估試驗品質與選取資料。

主要結論

搜尋到了2個合格的試驗。有1個針對130位評估為初次中風高危險群的鐮刀型貧血症病童(利用穿顱都卜勒超音波[transcranial doppler ultrasonography]),比較標準照護與維持鐮刀型血紅素(sickle haemoglobin)低於30%的長期輸血療法。此試驗中,有11位標準照顧組病童罹患中風,相較於輸血組的1位。輸血組的中風相對危險性(relative risk)相較於標準照顧組降低92%,注定此試驗要提早結束。原計畫治療30個月,但追蹤期間的中位平均為21.1個月。輸血組的併發症比率高:鐵負荷過高(iron overload)、異體免疫現象(alloimmunisation)與輸血反應。第2個試驗則是在輸血至少30個月後,評估中風的危險性。此試驗因停止輸血與繼續輸血(一般認為建議停止輸血不安全)的受試者間,出現中風風險的顯著差異而提早終止。中風風險是靠都卜勒超音波的異常流速來檢測,勝算比(OR)為0.02 (95% CI 0.00 to 0.43)。沒有找到研究輸血預防中風復發的試驗。

作者結論

這些試驗證明,常規輸血的受試者得到中風的風險顯著下降。一項追蹤試驗的資料指出,如果停止輸血,個體會回到原本的風險狀態。中風風險的程度,一定要和長期輸血的負擔間取得平衡。還需要進一步的研究,檢視利用輸血來預防次發性中風,並更清楚地界定中風的危險因子,以避免不必要地開始替病童輸血。

翻譯人

本摘要由臺灣大學附設醫院劉彥麟翻譯。

此翻譯計畫由臺灣國家衛生研究院(National Health Research Institutes, Taiwan)統籌。

總結

規則輸血可降低鐮刀型貧血症患者發生中風的危險,但有嚴重副作用。 鐮刀型貧血症造成紅血球下降(貧血)、組織氧氣減少和全身的問題。鐮刀型紅血球可阻塞腦血管病,並導致中風。本回顧發現,規常輸血可藉稀釋鐮刀型細胞,以降低初次和復發性中風的危險,但也有顯著的副作用,如:鐵負荷、經血液傳播的感染和輸血反應等。已在輸血的患者如停止輸血,反而會大幅增加中風的危險,可能與剛開始輸血時的危險性一樣高。