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重型β地中海贫血多次输血患者中的狼疮抗凝物和抗心磷脂抗体

Lupus anticoagulant and anticardiolipin antibodies in polytransfused beta thalassemia major.

作者信息

Sharma Sunita, Raina Vineeta, Chandra Jagdish, Narayan Shashi, Sharma Siddharth

机构信息

Department of Pathology, Lady Hardinge Medical College, New Delhi, India.

出版信息

Hematology. 2006 Aug;11(4):287-90. doi: 10.1080/10245330600954130.

DOI:10.1080/10245330600954130
PMID:17178669
Abstract

The currently used therapeutic strategies in beta thalassemia have prolonged the survival for many patients; this longer survival has been accompanied by the development of a number of unexpected complications, these include hemostatic derangements. The presence of anti-phospholipid antibodies (APA) (lupus anticoagulant, LA and anti-cardiolipid antibody, ACA) has recently been reported in polytransfused patients of beta thalassemia. In this study 50 patients with beta thalassemia major (beta-TM) who had received at least 20 transfusions were evaluated for presence of antiphospholipid antibodies and correlated with a number of clinical and hemostatic parameters. About 32% cases had developed a minor bleeding tendency and one also showed ecchymotic patch. None of the patients had thromboembolic episodes. LA was seen in 16% of cases. The mean age and number of transfusions were higher in LA positive patients as compared to LA negative patients however the results were not statistically significant. IgM ACA were detected in 6% of patients but no statistically significant correlation was found with age, number of transfusions, platelet count and coagulation parameters. IgG ACA were detected in 30% of cases and showed a significant correlation with number of blood transfusions (p = 0.016) and age (p = 0.031). Anti HCV antibodies were detected in 30% of patients out of which 1 had IgM ACA and 10 had IgG ACA. The latter showed a significant correlation with HCV infection. An increased incidence of LA and IgG ACA is found in polytransfused patients with beta-TM when compared to the normal healthy population but their clinical significance is yet not clearly understood.

摘要

目前用于β地中海贫血的治疗策略延长了许多患者的生存期;这种更长的生存期伴随着一些意想不到的并发症的出现,其中包括止血紊乱。最近有报道称,在接受多次输血的β地中海贫血患者中存在抗磷脂抗体(APA)(狼疮抗凝物、LA和抗心磷脂抗体、ACA)。在本研究中,对50例接受至少20次输血的重型β地中海贫血(β-TM)患者进行了抗磷脂抗体检测,并与一些临床和止血参数进行了相关性分析。约32%的病例出现轻微出血倾向,1例出现瘀斑。所有患者均无血栓栓塞事件。16%的病例检测到LA。与LA阴性患者相比,LA阳性患者的平均年龄和输血量更高,但结果无统计学意义。6%的患者检测到IgM ACA,但与年龄、输血量、血小板计数和凝血参数无统计学显著相关性。30%的病例检测到IgG ACA,且与输血量(p = 0.016)和年龄(p = 0.031)显著相关。30%的患者检测到抗HCV抗体,其中1例有IgM ACA,10例有IgG ACA。后者与HCV感染显著相关。与正常健康人群相比,接受多次输血的β-TM患者中LA和IgG ACA的发生率增加,但其临床意义尚不清楚。

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