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镰状细胞病和地中海贫血患者的同种免疫:阿曼一个中心的经验

Alloimmunization in Patients with Sickle Cell Disease and Thalassemia: Experience of a Single Centre in Oman.

作者信息

Alkindi Salam, AlMahrooqi Saba, AlHinai Sumaiya, AlMarhoobi Ali, Al-Hosni Saif, Daar Shahina, Fawaz Naglaa, Pathare Anil

机构信息

College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman; Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.

Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.

出版信息

Mediterr J Hematol Infect Dis. 2017 Feb 15;9(1):e2017013. doi: 10.4084/MJHID.2017.013. eCollection 2017.

Abstract

BACKGROUND

Blood transfusion is an integral part of the supportive care for patients with sickle cell disease (SCD) and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy.

OBJECTIVES

The aim of this study was to evaluate the prevalence of red cell alloimmunization in Omani patients with sickle cell anaemia and thalassemia.

METHODS

This study included 262 patients whose historical transfusion records were available. One hundred and twenty-nine patients with thalassaemia who were attending the day care unit for regular transfusions, and 133 SCD patients admitted at our hospital were included in this study. The Diamed® gel system was used for the screening and identification of atypical antibodies.

RESULTS

The rate of alloimmunization in SCD patients was 31.6% (n=42, 95%CI, 24.87-40.66), whereas in patients with thalassaemia it was 20% (n=26; 95%CI, 13.9-27.6). Antibodies to E, e, C, c, D, K, S, Fyª, Kpª, Jkª and C were observed; 85% of the patients were also immunised with Rh and Kell antigens. Considering the two groups together, 8 developed nonspecific antibodies and 12 developed more than one antibody.

CONCLUSIONS

Red cell transfusions were associated with a significant risk of alloimmunization. It is, therefore, imperative to perform an initial extended red cell phenotyping for both donors and recipients, and carefully select ABO, Rh and Kell matched donors. The higher incidence of alloimmunization in SCD patients is related to the inherent SCD-specific inflammatory state.

摘要

背景

输血是镰状细胞病(SCD)和地中海贫血患者支持性治疗的一个组成部分。然而,红细胞同种免疫的风险是这种治疗的主要并发症之一。

目的

本研究的目的是评估阿曼镰状细胞贫血和地中海贫血患者中红细胞同种免疫的患病率。

方法

本研究纳入了262例有既往输血记录的患者。其中包括129例在地日间护理单元接受定期输血的地中海贫血患者,以及133例在我院住院的SCD患者。使用Diamed®凝胶系统进行非典型抗体的筛查和鉴定。

结果

SCD患者的同种免疫率为31.6%(n = 42,95%CI,24.87 - 40.66),而地中海贫血患者的同种免疫率为20%(n = 26;95%CI,13.9 - 27.6)。观察到针对E、e、C、c、D、K、S、Fyª、Kpª、Jkª和C的抗体;85%的患者也对Rh和Kell抗原产生了免疫。将两组患者合并考虑,8例患者产生了非特异性抗体,12例患者产生了不止一种抗体。

结论

红细胞输血与显著的同种免疫风险相关。因此,对供体和受体进行初始扩展红细胞表型分析并仔细选择ABO、Rh和Kell匹配的供体是至关重要的。SCD患者中同种免疫发生率较高与SCD固有的特异性炎症状态有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fac0/5333756/1f7819e4983d/mjhid-9-1-e2017013f1.jpg

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