Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease

 

Abstract

Background

Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world.

Objectives

To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions.

Search methods

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and handsearching relevant journals and abstract books of conference proceedings.

Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.

Date of the most recent search: 27 August 2010.

Selection criteria

All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises with no treatment or blood transfusions in people with sickle cell disease.

Data collection and analysis

No trials of splenectomy for acute splenic sequestration were found.

Main results

No trials of splenectomy for acute splenic sequestration were found.

Authors' conclusions

Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises. However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. There is a need for a well-designed, adequately-powered, randomized controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes, as a means of improving survival and decreasing mortality from acute splenic sequestration in people with sickle cell disease.

 

Plain language summary

Removing spleens from people with sickle cell disease after a splenic sequestration compared to treating them with blood transfusions to prevent further attacks

In some people with sickle cell disease, red blood cells become trapped and destroyed in the spleen. This damages the spleen, which may become enlarged leading to splenic sequestration crises. These crises consist of abdominal pain, rapid heart rate and other symptoms. Such an attack can be fatal without prompt treatment. All or part of the spleen (splenectomy) is often removed after a person has survived such a crisis to try and prevent another one. This surgery may leave the individual at a higher risk of infection. We looked for trials which compared surgery to blood transfusions. We found no trials to provide reliable evidence about the risks or benefits of splenectomy for people with sickle cell disease after splenic sequestration. There is a need for a trial to assess the benefits and risks of splenectomy compared to transfusion programmes.

 

摘要

背景

鐮刀型貧血症(sickle cell disease)患者的急性脾臟隔斷危象(acute sequestration crises)採用脾臟切除術(splenectomy)與保守治療的比較

急性脾臟隔斷危象是鐮刀型貧血症的一種併發症，死亡率與復發率都高。脾臟切除術和輸血及其後續治療，是世界各地長期照護的主流。

目標

評估鐮刀型貧血症患者接受(全部或部分)脾臟切除術，與常規輸血相比，能否預防急性脾臟隔斷危象、改善存活率、降低併發症。

搜尋策略

我們搜尋了Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies 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學術會議摘要集所組成。由這些試驗與回顧的參考書目中，利用此搜尋策略，還尋找了其他的試驗。最近搜尋時間：2006年11月。

選擇標準

針對鐮刀型貧血症患者，所有比較(全部或部分)脾臟切除術與無治療或輸血以預防急性脾臟隔斷復發的隨機或半隨機對照試驗(randomised or quasirandomised controlled trials)。

資料收集與分析

沒有找到脾臟切除術用於急性脾臟隔斷的試驗。

主要結論

沒有找到脾臟切除術用於急性脾臟隔斷的試驗。

作者結論

全部脾臟切除術能預防進一步隔斷，部分切除則可能可以降低急性脾臟隔斷危象復發的風險。然而，尚缺乏試驗提供證據以證明鐮刀型貧血症患者接受脾臟切除術可改善存活率、降低併發症。有需要進行設計良好、強度充分的隨機對照試驗(randomized controlled trial)，以在能否改善存活率、降低急性脾臟隔斷死亡率等方面，評估鐮刀型貧血症患者接受脾臟切除術與輸血療法相比的好處與風險。

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

鐮刀型貧血症患者發生脾臟隔斷後，切除脾臟可預防再度發作，但我們無法找到有評估其他治療預後的試驗。在某些鐮刀行貧血症患者，紅血球會在脾臟內堆積並被破壞，進而損傷脾臟，使其腫大並引起脾臟隔斷(一種合併腹痛、心跳加速和其他症狀的發作)，若不及時治療可能致命。隔斷發作後的存活者，往往會接受脾臟的部份或完全切除(脾臟切除術)，以預防再度發作，但此作法可能為患者帶來高感染風險。關於鐮刀型貧血症患者出現脾臟隔斷後接受脾臟切除術，我們找不到能提供可靠證據的試驗。