Islam M A, Alam F, Gan S H, Sasongko T H, Wan Ghazali W S, Wong K K
Universiti Sains Malaysia, School of Medical Sciences, Human Genome Centre, Kelantan, Malaysia.
Malays J Pathol. 2017 Aug;39(2):123-133.
Antiphospholipid syndrome (APS) is an autoimmune disorder characterised by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPLs) based on the Sydney criteria. We aimed to explore the clinico-laboratory features and treatment strategies of APS patients retrospectively.
The medical records of APS patients registered under Hospital Universiti Sains Malaysia (Kelantan state) between 2000 and 2015 were reviewed.
A total of 17 APS subjects (age 40.7 ± 12.8 years) including 11 primary (64.7%) and six secondary APS (35.3%) patients were identified. The follow-up period was 9.5 ± 6.7 years with male:female ratio of 1.0:4.7. Pregnancy morbidity was the most common clinical manifestation (11/14; 78.6%) followed by recurrent venous thrombosis (10/17; 58.8%). For other clinical features, menorrhagia was the most frequently observed manifestation (4/14; 28.6%) followed by aPLs-associated thrombocytopenia (4/17; 23.5%) and ovarian cyst (3/14; 21.4%). LA and aCL were positive in 94.1% (16/17) and 81.8% (9/11) of the patients, respectively. APTT value (76.7 ± 17.0 sec) was significantly high (p < 0.05). Low intensity warfarin alone was successful to maintain target INR (2.0 - 3.0) and prevent recurrence of thrombosis.
The tendency of pregnancy morbidity in this cohort of Malaysian Kelantanese APS patients was high compared to other previously reported APS cohorts. Low intensity warfarin was successful in preventing recurrence of thrombosis, however, APS women receiving long-term anticoagulants should be monitored for possible occurrence of menorrhagia and ovarian cysts.
抗磷脂综合征(APS)是一种自身免疫性疾病,根据悉尼标准,其特征为在存在抗磷脂抗体(aPLs)的情况下出现血栓形成和/或妊娠并发症。我们旨在回顾性探讨APS患者的临床实验室特征及治疗策略。
回顾了2000年至2015年间在马来西亚理科大学医院(吉兰丹州)登记的APS患者的病历。
共确定了17例APS患者(年龄40.7±12.8岁),其中包括11例原发性(64.7%)和6例继发性APS(35.3%)患者。随访期为9.5±6.7年,男女比例为1.0:4.7。妊娠并发症是最常见的临床表现(11/14;78.6%),其次是复发性静脉血栓形成(10/17;58.8%)。对于其他临床特征,月经过多是最常观察到的表现(4/14;28.6%),其次是aPLs相关血小板减少症(4/17;23.5%)和卵巢囊肿(3/14;21.4%)。狼疮抗凝物(LA)和抗心磷脂抗体(aCL)分别在94.1%(16/17)和81.8%(9/11)的患者中呈阳性。活化部分凝血活酶时间(APTT)值(76.7±17.0秒)显著升高(p<0.05)。单独使用低强度华法林成功维持了目标国际标准化比值(INR)(2.0 - 3.0)并预防了血栓形成的复发。
与其他先前报道的APS队列相比,这一马来西亚吉兰丹州APS患者队列中妊娠并发症的倾向较高。低强度华法林成功预防了血栓形成的复发,然而,接受长期抗凝治疗的APS女性应监测月经过多和卵巢囊肿的可能发生情况。