红细胞及血小板减少或全血细胞减少孕妇的临床分析

Clinical analysis of bicytopenia and pancytopenia during pregnancy

目的探讨妊娠期红细胞及血小板（两系）减少或全血细胞减少的诊断与治疗、妊娠结局及预后。方法收集2002年5月-2008年5月北京大学人民医院收治的24例妊娠期首次发现两系或全血细胞减少孕妇的临床资料，同顾性分析其诊断、治疗经过及妊娠结局，随访其预后情况。结果（1）诊断：根据临床及实验窒检查（主要包括血常规检查及网织红细胞计数、外周血涂片检查、血清叶酸及维生素B12水平测定、自身免疫抗体筛查、骨髓穿刺涂片及活检），24例患者中13例诊断为慢性再生障碍性贫血（CAA），6例诊断为骨髓增生异常综合征（MDS），2例诊断为巨幼细胞性贫血（MA），阵发性睡眠性血红蛋白尿（PNH）、Evan综合征和急性白血病各1例。（2）治疗及妊娠结局：孕期以积极的支持疗法和间断输血为主。6例出现妊娠并发症，其中重度子痫前期4例（同时伴有颅内出血及胎死宫内各1例），妊娠期糖尿病2例。21例患者妊娠至足月或近足月分娩，新生儿结局良好。（3）预后：产后对患者进行r为期2个月～4年的随访，其中2例MA患者血常规检查恢复正常；PNH患者和Evan综合征患者分别为轻度贫血及轻度m小板减少；急性白血病患者产后半年因复发而死亡；13例CAA患者中，血常规检查完全正常2例，部分正常6例，仍为异常4例，失访1例；6例MDS患者中1例血常规检查部分止常，4例仍为异常，1例转化为急性单核细胞性白血病，因拒绝化疗而失访。结论CAA是妊娠期两系或伞血细胞减少的最常见原因，其次为MDS。多数患者经积极的支持疗法，妊娠结局良好，但由于病因不同，患者的预后存存差异。

Objective To investigate the diagnosis, management, pregnancy outcome and prognosis of bicytopenia or pancytopenia during pregnancy. Methods Retrospective chart review was conducted on 24 pregnancies who were found bicytopenia or pancytopenia during pregnancy for the first time. The diagnoses were reconfirmed. The management and pregnancy outcome were collected. And the prognoses were followed. Results According to the clinical data and laboratory findings, the latter including complete blood cell count, reticulocyte count, peripheral smear, serum folate and vitamin Bj2 level, autoimmune antibody screening, bone marrow smear and biopsy, thirteen patients were diagnosed as having chronic aplastic anemia （CAA）, six as having myelodysplastic syndromes （MDS）, two as having megaloblastic anemia（ MA）, one as having paroxysmal nocturnal hemoglobinuria（PNH）, one as having Evan＇s syndrome and one as having acute leukemia. The management basically consisted of supportive transfusions. Six patients suffered pregnancy complications including four with severe preeclampsia （one with intracranial hemorrhage and one with intrauterine death concomitantly） and two with gestational diabetes. The delivery ages of the 21 patients were term or nearly term with all good neonatal outcomes. Postpartum follow-up showed the two patients with MA achieved complete remission, the one with PNH had mild anemia and that with Evan＇s syndrome had mild thrombocytopenia. The patient with acute leukemia died of recurrence six months postpartum. Of the thirteen patients with CAA, two achieved complete remission, six partial remission, four no remission and one was lost follow-up. Of the 6 patients with MDS, one achieved partialremission, four no remission, and one transformed into acute monocytic leukemia, then refused chemotherapy and was lost follow-up. Conclusions CAA may be one of the most common causes of bicytopenia or pancytopenia during pregnancy, MDS may be the second. Diagnosis should be made as soon as possible through appropriate and reasonable laboratory examinations. Most patients could achieve good pregnancy outcomes through supportive management. The maternal prognosis may vary widely depending on the causes.