妊娠合并极重度血小板减少26例临床分析

目的 探讨妊娠合并极重度血小板减少患者的病因诊断、临床处理及妊娠结局.方法 回顾性分析2004年1月到2009年3月在北京大学人民医院产科分娩的26例妊娠合并极重度血小板减少患者的临床资料.以孕期至少有2次或2次以上血小板计数<10×109/L为极重度血小板减少诊断标准.进行病因诊断,根据不同病因给予以下临床处理.(1)孕期在无全身自发性出血情况下,维持血小板计数>20×109/L,血红蛋白>70 g/L;(2)血小板<10×109/L或有出血倾向时,输注血小板;红细胞压积(HCT)<25%,血红蛋白<70 g/L时,输注压积红细胞;(3)剖宫产术前或分娩前维持血红蛋白>70 g/L,血小板计数>30×109/L;(4)特发性血小板减少性紫癜(ITP)患者血小板<(20～30)×109/L或有出血倾向时,用泼尼松或联合丙种球蛋白治疗.对于上述治疗无效者,若血小板<10×109/L或有出血倾向时间断输注血小板,无出血倾向者根据孕周严密观察,适时终止妊娠.对患者的一般临床资料、病因诊断、临床处理及妊娠结局进行分析.结果 (1)发病率及发病原因:同期分娩数为9302例,妊娠合并极重度血小板减少患者26例,妊娠合并极重度血小板减少的发生率为0.28%.患者年龄平均29岁.孕前诊断17例,孕期诊断9例.26例患者中,有13例在我院行系统产前检查,初次诊断极重度血小板减少时的平均孕周为24周;无系统产前检查的13例,初次诊断极重度血小板减少时的平均孕周为32周.26例患者中,2例(8%,2/26)病因诊断不明,24例(92%,24/26)病因诊断明确,其中14例(54%,14/26)为ITP,5例为骨髓增生异常综合征(MDS),4例为慢性再生障碍性贫血(CAA),1例为系统性红斑狼疮(SLE).(2)临床处理:所有患者均多次应用血制品.14例ITP患者中6例应用了泼尼松+丙种球蛋白治疗,8例仅应用泼尼松治疗.26例患者中有9例(35%,9/26)出现了妊娠并发症,其中6例(6/9)并发子痫前期,2例发生妊娠期糖尿病,1例为弥漫性肺泡出血.26例患者平均分娩孕周为36周,阴道分娩3例,平均出血量为83 ml;23例剖宫产分娩,平均出血量为410 ml.(3)围产儿结局:26例围产儿中,1例胎死宫内,25例活婴,其中12例为早产儿.平均孕龄36周,平均出生体质量2877 g.2例ITP患者分娩的新生儿出现了重度血小板减少.结论 妊娠合并极重度血小板减少的主要病因是ITP,治疗以泼尼松+丙种球蛋白为主;其次是CAA和MDS,以支持疗法为主.妊娠合并极重度血小板减少并非终止妊娠的绝对指征,可根据不同病因予相应治疗后,在严密的围产期监测下,注意防治妊娠并发症,分娩方式以剖宫产为主,可获得较好的妊娠结局.

Clinical analysis of pregnancy complicated with severe thrombocytopenia

Objective To investigate the etiology and perinatal outcome of pregnancies complicated with extremely severe thrombocytopenia [ at least two times of platelets count (PLT) < 10 × 109/L during pregnancy]. Methods Clinical data, including basic information, etiology, management and outcomes of pregnant women with extremely severe thrombocytopenia, admitted to Peking University People's Hospital from January 2004 to March 2009, were retrospectively collected. The management of these cases varied according to different etiology and the symptoms: (1) PLT were maitained > 20 × 109/L and hemoglobulin> 70 g/L in those women without spontaneous bleeding; (2) PLT transfusion would be required when PLT< 10 × 109/L or bleeding occur and RBC would be supplied when hematocrit <25% and hemoglobulin <70g/L; (3) Hemoglobulin should be > 70 g/L and PLT >30 × 109/L before cesarean section or delivery;(4) Predinisone and/or intravenous immunoglobulin G (IVIG) would be given in women complicated with idiopathic thrombocytopenic purpura (ITP) when PLT < (20-30) × 109/L or bleeding. PLT would be given if all the above management were failed, or PLT < 10 × 109/L, or bleeding. Women without bleeding would be closely monitored and delivery would be planned. Results (1) Twenty-six cases were identified among 9302 deliveries during the study period (0.28%), with an average of maternal age of 29. Seventeen were diagnosed before conception and 9 during pregnancy. Among the 26 women, half received regular prenatal check in our hospital and the average gestations at diagnosis was 24 weeks and the other half without regular prenatal visits and the average gestations at diagnosis was 32 weeks. Etiology was identified in 24 out of the 26 women, including 14(54%) ITP, 5 myelodysplastic syndrome (MDS), 4 chronic aplastic anaemia(CAA) and 1 systemic lupus erythematosus (SLE). (2) Management: All of the 26 women received blood products. Among the 14 ITP cases, 6 received predinisone and IVIG and 8 only took predinisone. Nine of the 26 patients (35%) had pregnant complications, among which 6 (6/9) were preeclampsia. The overall average gestation at delivery was 36 weeks. Only 2 delivered vaginally with the average blood loss of 83 ml and 23 cesarean sections were performed with the average blood loss of 410 ml. (3) Perinatal outcomes:There were 26 perinatal babies, among which 1 died intrauterine and 25 were born alive (12 preterm infants). The average birth weight was 2877 g. Neonatal severe thrombocytopenia presented in 2 newborns whose mother complicated with ITP. Conclusions The main cause of extremely severe thrombocytopenia during pregnancy is ITP, managed mainly by predinisone and IVIG, followed by CAA and MDS, which may require supportive treatment. Pregnancy complicated with extremely severe thrombocytopenia is not an indication of termination. Better maternal and fetal outcomes can be achieved through proper treatment based on the etiology, intensive care in prevention and management of complications and cesarean section.