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. 2016 May 19;2016(5):CD007001.
doi: 10.1002/14651858.CD007001.pub4.

Hematopoietic stem cell transplantation for people with sickle cell disease

Chioma Oringanje  1 , Eneida Nemecek, Oluseyi Oniyangi
Affiliations

Hematopoietic stem cell transplantation for people with sickle cell disease

Chioma Oringanje et al. Cochrane Database Syst Rev. .

Update in

Abstract

Background: Sickle cell disease is a genetic disorder involving a defect in the red blood cells due to its sickled hemoglobin. The main therapeutic interventions include preventive and supportive measures. Hematopoietic stem cell transplantations are carried out with the aim of replacing the defective cells and their progenitors (hematopoietic (i.e. blood forming) stem cells) in order to correct the disorder. This is an update of a previously published review.

Objectives: To determine whether stem cell transplantation can improve survival and prevent symptoms and complications associated with sickle cell disease. To examine the risks of stem cell transplantation against the potential long-term gain for people with sickle cell disease.

Search methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Group's Haemoglobinopathies Trials Register complied from electronic searches of the Cochrane Central Register of Controlled Trials (CENTRAL) (updated each new issue of The Cochrane Library) and quarterly searches of MEDLINE.Unpublished work was identified by searching the abstract books of major conference proceedings and we conducted a search of the website: www.ClinicalTrials.gov.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 06 October 2015.

Selection criteria: Randomized controlled and quasi-randomized studies that compared any method of stem cell transplantation with either each other or with any of the preventive or supportive interventions (e.g. periodic blood transfusion, use of hydroxyurea, antibiotics, pain relievers, supplemental oxygen) in people with sickle cell disease irrespective of the type of sickle cell disease, gender and setting.

Data collection and analysis: No relevant trials were identified.

Main results: Ten trials were identified by the initial search and none for the update. None of these trials were suitable for inclusion in this review.

Authors' conclusions: Reports on the use of hematopoietic stem cell transplantation improving survival and preventing symptoms and complications associated with sickle cell disease are currently limited to observational and other less robust studies. No randomized controlled trial assessing the benefit or risk of hematopoietic stem cell transplantations was found. Thus, this systematic review identifies the need for a multicentre randomized controlled trial assessing the benefits and possible risks of hematopoietic stem cell transplantations comparing sickle status and severity of disease in people with sickle cell disease.

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Conflict of interest statement

Chioma Oringanje: none known. Eneida Nemecek: none known. Oluseyi Oniyangi: none known.

Update of

References

References to studies excluded from this review

Bernaudin 2007 {published data only}
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Hongeng 2004 {published data only}
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Horan 2005 {published data only}
    1. Horan JT, Liesveld JL, Fenton P, Blumberg N, Walters MC. Hematopoietic stem cell transplantation for multiply transfused patients with sickle cell disease and thalassemia after low‐dose total body irradiation, fludarabine, and rabbit anti‐thymocyte globulin. Bone Marrow Transplantation 2005;35(2):171‐7. - PubMed
Iannone 2003 {published data only}
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Locatelli 2003 {published data only}
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Vermylen 2000 {published data only}
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References to other published versions of this review

Oringanje 2009
    1. Oringanje C, Nemecek E, Oniyangi O. Hematopoietic stem cell transplantation for children with sickle cell disease. Cochrane Database of Systematic Reviews 2009, Issue 1. [DOI: 10.1002/14651858.CD007001.pub2] - DOI - PubMed
Oringanje 2013
    1. Oringanje C, Nemecek E, Oniyangi O. Hematopoietic stem cell transplantation for people with sickle cell disease. Cochrane Database of Systematic Reviews 2013, Issue 5. [DOI: 10.1002/14651858.CD007001.pub3] - DOI - PubMed

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