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非洲喀麦隆血友病患者中的凝血因子VIII和IX抑制剂:一项初步研究。

FVIII and FIX inhibitors in people living with hemophilia in Cameroon, Africa: a preliminary study.

作者信息

Balôgôg P N, Tagny C T, Ndoumba A, Mbanya D

出版信息

Int J Lab Hematol. 2014 Oct;36(5):566-70. doi: 10.1111/ijlh.12190.

DOI:10.1111/ijlh.12190
PMID:25356499
Abstract

INTRODUCTION

Hemophilia is a reality in Cameroon were more than 100 patients are registered and followed-up in the Yaounde Hemophilia treatment center.

AIM

We conducted a cross sectional study in patient living with hemophilia in that setting in order to determine the frequency of Factor VIII and IX specific inhibitors and the factors associated their presence.

METHODS

From May to December 2012, venous blood samples of hemophiliac patients undergoing replacement therapy were collected in tubes containing 0.109 M (3.2%) trisodium citrate. Inhibitors were screened by APTT based method using 50 : 50 patients' plasma mixed with normal plasma incubated together for 2 h at 37 °C. Quantitative assay was carried out to measure Bethesda units (BU) with the Bethesda Nijmegen Assay. Samples were labeled as low titer inhibitor when <5 BU were detected.

RESULTS

A total of 42 patients were tested among which four had haemophilia B while 38 had hemophilia A. Eight patients (19%) were found to have positive Bethesda test. The mean age of patients with inhibitors was 9.2 ± 6 years (2-18). Two of them were high responders (25%). the frequency of inhibitors was higher in severe hemophiliac patients.

CONCLUSION

The frequency of inhibitors in hemophiliac patient undergoing replacement therapy is high in Cameroon. Severity of disease was found to be the main contributing factor in patients who developed inhibitors.

摘要

引言

在喀麦隆,血友病是一个现实情况,雅温得血友病治疗中心登记并随访了100多名患者。

目的

我们对该地区血友病患者进行了一项横断面研究,以确定凝血因子VIII和IX特异性抑制剂的发生率及其存在的相关因素。

方法

2012年5月至12月,将接受替代疗法的血友病患者的静脉血样本采集到含有0.109 M(3.2%)柠檬酸钠的试管中。使用患者血浆与正常血浆按50:50混合在37℃孵育2小时的基于活化部分凝血活酶时间(APTT)的方法筛查抑制剂。采用贝塞斯达尼美根试验进行定量测定以测量贝塞斯达单位(BU)。当检测到<5 BU时,样本被标记为低滴度抑制剂。

结果

共检测了42名患者,其中4名患有B型血友病,38名患有A型血友病。8名患者(19%)贝塞斯达试验呈阳性。有抑制剂的患者平均年龄为9.2±6岁(2 - 18岁)。其中2名是高反应者(25%)。重度血友病患者中抑制剂的发生率更高。

结论

在喀麦隆,接受替代疗法的血友病患者中抑制剂的发生率很高。发现疾病严重程度是发生抑制剂的患者的主要促成因素。

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