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目的 探讨妊娠期首发血小板减少的病因学诊断依据及临床特点.方法 选择2000年1月到2010年1月间北京大学人民医院产科收治的妊娠期血小板减少的孕妇,纳入标准以血小板减少为妊娠期首发临床症状,妊娠期有两次或以上血小板计数＜ 100×109/L,其中资料完整、具有明确随访结局和病因学诊断的159例纳入本研究.本院同期分娩总数为16 562例,发生率为0.96％.对159例患者的一般资料、妊娠期发病时间、血小板计数变化、相关检查、母儿围产期情况进行分析,产后进行随访.结果 (1)病因诊断:159例患者妊娠期血小板减少的病因学诊断结果为妊娠期血小板减少症(GT) 101例(63.5％),特发性血小板减少性紫癜(ITP) 43例(27.0％),血液系统疾病9例[5.7％,巨幼细胞性贫血(MA)4例、再生障碍性贫血(AA)2例、骨髓异常增生综合征(MDS)3例],免疫系统疾病6例[3.8％,系统性红斑狼疮(SLE)3例、抗磷脂综合征(APS)2例、Evans综合征1例].(2)母儿围产期情况:159例患者中,并发妊娠期高血压疾病21例(13.2％),糖代谢异常13例(8.2％),贫血44例(27.7％),早产18例(11.3％);妊娠期给予糖皮质激素或丙种球蛋白治疗29例(18.2％).终止妊娠平均孕38周.阴道分娩55例(34.6％),剖宫产104例(65.4％).产后出血34例(21.4％),产褥感染2例(1.3％),无孕产妇死亡.围产儿总数160例(1例双胎),分娩活产儿157例,胎死宫内3例,早期新生儿死亡2例,胎儿生长受限4例,新生儿血小板减小6例,无颅内出血发生.(3)血小板减少的发病时间:159例患者中,妊娠早期、中期、晚期出现血小板减少者分别为29例(18.2％)、67例(42.1％)、63例(39.6％).GT与1TP在发病时间上比较,差异有统计学意义(P＜0.05);GT趋向于妊娠中、晚期发病,ITP趋向于妊娠早、中期发病.(4)血小板减少的程度:妊娠期血小板最低程度在(51 ～ 100)×109/L、(31 ～50)×109/L、( 10～ 30)×109/L、＜10×109/L范围内者分别为75例(47.2％)、39例(24.5％)、31例(19.5％)、14例(8.8％).GT与ITP在血小板降低程度上比较,差异有统计学意义(P＜0.01).(5)合并妊娠期贫血的情况:159例患病者中合并妊娠期贫血者共44例(27.7％),其中GT 10例(9.9％,10/101),ITP 25例(58.1％,25/43),血液系统疾病5例(5/9),免疫系统疾病(Evans综合征)1例(1/6).GT与ITP在合并贫血发生率方面比较,差异有统计学意义(P＜0.01).(6)产后血小板计数恢复情况:对159例患者从产后开始至10年进行了随访,产后1周内、6周内、半年内血小板计数恢复正常者分别为66例(41.5％)、43例(27.0％)、17例(10.7％),半年以上未恢复者33例(45.7％).结论 妊娠期首发血小板减少的主要病因为GT,其次为ITP,两者妊娠期发病时间、血小板计数降低的程度、合并贫血及产后血小板计数恢复情况均有所不同;少见病因包括部分免疫及血液系统疾病.临床应重视通过相关检查进行病因学诊断及鉴别.

Objective To investigate the etiology and clinical characteristics of pregnancy-emerged thrombocytopenia.Methods A retrospective analysis was conducted on clinical data of 159 pregnancies with thrombocytopenia,who were admitted to Peking University People's Hospital from January 2000 to January 2010.All the patients recruited in this study had no history of blood or immune system disease before pregnancy,and thrombocytopenia was the predominate clinical manifestation during pregnancy,with platelet counts less than 100 × 109/L at least twice during pregnancy.The thrombocytopenia should not be induced by drugs,viral infections,preeclampsia or hemolysis,elevated liver enzymes,and low platelets syndrome (HELLP).All cases were followed up.The general condition,the onset time of thrombocytopenia,platelet changes,accompany symptoms,maternal and perinatal outcomes as well as follow-up conditions were compared based on the etiology.Results ( 1 ) Etiology:among the 159 cases,101 (63.5％) were diagnosed gestational thrombocytopenia (GT) ;43 ( 27.0％ ) were idiopathic thrombocytopenic purpura(ITP) ;9 ( 5.7％ ) were blood system diseases,including 4 cases of megaloblastic anemia( MA ),2 cases of aplastic anaemia (AA),and 3 cases of myelodysplastic syndrome(MDS).Six cases (3.8％)were diagnosed immune system diseases,including 3 cases of systemic lupus erythematosus ( SLE),2 cases of antiphospholipid syndrome (APS),and 1 case of Evans syndrome.(2)Maternal and perinatal outcomes:pregnancy induced hypertension was diagnosed in 21 cases ( 13.2％ ),abnormal glucose metabolism in 13 cases ( 8.2％ ),anemia in 44 cases ( 27.7％ ) and preterm delivery in 18 cases ( 11.3％ ).Twenty-nine cases ( 18.2％ ) were treated with corticosteroids or gamma globulin during pregnancy.The average gestational week was 38 weeks.Fifty-five cases ( 34.6％ ) underwent vaginal delivery,104 cases ( 65.4％ ) received cesarean section.Postpartum hemorrhage was observed in 34 cases (21.4％),and puerperal infection happened in 2 eases ( 1.3％ ).No maternal death was found.In a total of 160 fetuses (including twins),there were 157 live births.Three cases of fetal death and 2 cases of early neonatal deaths were observed.Fetal growth restriction was observed in 4 cases,and neonatal thrombocytopenia was seen in 6 cases.No intracranial hemorrhage was detected.(3)The onset time of thrombocytopenia:among the 159 cases,29 cases ( 18.2％ ),67 cases (42.1％ ),63 cases (43.6％) of thrombocytopenia were detected in the first,second and third trimester,respectively.There was a significant difference of the onset time of thrombocytopenia between GT and ITP groups( P ＜ 0.05 ).Patients with GT tended to have a later onset of thrombocytopenia,which mainly happened in the second and third trimester,while patients with ITP tended to happen in the first and second trimester.(4)The degree of thrombocytopenia:the cases with the minimum platelets level of (51 - 100) × 109/L,(31 - 50) × 109/L,( 10 - 30) × 109/L,＜ 10 × 109/L during pregnancy were 75 (47.2％ ),39 (24.5％ ),31 ( 19.5％ ),14( 8.8％ ) respectively.There was a significant difference between GT and ITP groups in the lowest platelets level (P ＜ 0.01 ).(5)Thrombocytopenia accompany with anemia:among the 159 cases,there were 44 cases (27.7％ ) accompanied with anemia.The proportion was 9.9％ ( 10/101 ) in GT group,58.1％ (25/43) in ITP group,with significant difference(P ＜0.01 ).Anemia was also found in 5 cases in blood system disease group (5/9),and 1 case in immune system disease group (Evans syndrome,1/6).Pancytopenia was observed in 2 cases with ITP (4.7％,2/43 ) and 3 cases with blood system disease ( AA:1 cases,MA:2 cases,3/9).(6) The recovery of the platelets counts postpartum:the postpartum follow-up periods were 7 months to 10 years.Patients recovered within 1 week,6 weeks,6 months postpartum were 66 cases ( 41.5％ ),43 cases ( 27.0％ ),17 cases ( 10.7％ ) respectively.The platelets counts did not recover within 6 months postpartum in 33 cases(45.7％ ).Conclusions GT is the leading cause of pregnancy-emerged thrombocytopenia followed by ITP.There are significant differences between GT and ITP in the onset time of thrombocytopenia,the lowest platelets level,the proportion of anemia accompanied and the postpartum recovery.Other etiologies including immune and blood system diseases are rare.The relevant examinations should be taken for etiology and differential diagnosis.