妊娠合并原发性血小板增多症的治疗及母儿结局

Clinical analysis of essential thrombocythemia and pregnancy： treatment and outcomes

目的 探讨妊娠合并原发性血小板增多症患者妊娠期适宜的临床干预手段.方法 回顾性分析2005年3月至2015年1月经北京大学人民医院产科、血液内科联合诊治的18例妊娠合并ET患者共22次妊娠的临床资料,对诊疗经过、妊娠结局及产后随访情况进行分析.采用秩和检验、x2检验或Fisher＆#39;s精确概率法等进行统计学分析.结果 （1）22次妊娠的活产率为77％（17/22）,妊娠丢失率为23％（5/22）,其中自然流产4次;（2）6次妊娠未接受任何治疗;16次妊娠孕前和（或）孕期接受临床治疗,包括单独或联合使用小剂量阿司匹林口服、羟基脲口服、干扰素α皮下注射及血小板单采术;（3）与无任何治疗相比,孕前和/或妊娠期治疗干预可显著提高活产率（15/16与2/6,P=0.009）,新生儿随访至今血小板计数均正常;（4）单纯口服小剂量阿司匹林患者治疗前中位血小板水平为761×109/L[（448～1 213）×109/L],治疗结束时为639×109/L[（283～1 908）×109/L],差异无统计学意义（Z=-0.405,P=0.686）;（5）口服小剂量阿司匹林联合干扰素α皮下注射患者治疗前中位血小板水平为989×109/L[（435～1 504）×109/L],治疗结束时为498×109/L[（186～559）×109/L],出现显著下降（Z=-2.366,P=0.018）.结论 （1）妊娠早期自然流产是妊娠合并原发性血小板增多症最常见的并发症;（2）临床干预可改善妊娠合并原发性血小板增多症患者的妊娠结局,且尚无远期不良影响;（3）妊娠期干扰素α联合小剂量阿司匹林可能是目前较为适宜的妊娠合并原发性血小板增多症的临床干预措施.

Objectives To summarize the maternal and fetal outcomes of patients with essential thrombocythemia （ET）, and tentatively propose possible solutions for different clinical scenarios.Methods We retrospectively studied the clinical data, treatment, outcomes and follow-up status in 18 consecutive cases of young women with ET visited Peking University People＆#39;s Hospital, among whom 22 pregnancies occurred from March 2005 to January 2015.Rank-sum test, Chi-square test or Fisher exact test were applied for statistics.Results （1） Out of the total 22 pregnancies, 17 （77%） were successful （term live births） and five （23%） ended in miscarriages, among which four were spontaneous abortion in first trimester.（2） Six pregnancies did not received any therapy, while 16 received various treatments before and/or during pregnancy, including oral Aspirin（single/joint）, Hydroxyurea, Interferon-α （IFN-α） and platelet apheresis.（3） Treatments before/during pregnancy improved the live birth rate significantly （15/16 vs 2/6, P=0.009）.Platelet （PLT） count of the newborns were all normal till the time at follow-up.（4） The median PLT count in those patients before Aspirin therapy was 761 × 109/L[（448-1 213） × 109/L, and 639× 109/L[（283-1 908） × 109/L] thereafter, which showed no significant difference （Z=-0.405, P=0.686）.（5） The PLT counts in those patients before combination therapy of Aspirin and IFN-α was 989 × 109/L[（435-1 504） × 109/L], and 498 × 109/L[（186-559） × 109/L] thereafter, which decreased significantly （Z=-2.366, P=0.018）.Conclusions （1） First-trimester spontaneous abortion is the most common complication of ET in pregnancy.（2） A specific treatment strategy may improve the pregnant outcomes without long-term harmful effect up to now.（3） IFN-α and/or Aspirin might be the first-line therapy in women with ET during pregnancy.