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妊娠相关性再生障碍性贫血——附10例病例报告并文献复习

Pregnancy associated aplastic anemia--a series of 10 cases with review of literature.

作者信息

Choudhry V P, Gupta Sunil, Gupta Meenal, Kashyap Rajesh, Saxena R

机构信息

Department of Hematology, All India Institute of Medical Sciences, New Delhi 110029, India.

出版信息

Hematology. 2002 Aug;7(4):233-8. doi: 10.1080/1024533021000024067.

DOI:10.1080/1024533021000024067
PMID:14972785
Abstract

INTRODUCTION

Pregnancy induced aplastic anemia is a rare entity and the association is not well explained. There are approximately 80 cases in the literature and we are presenting the largest series, so far, of 10 cases.

RESULTS

Total of 10 cases had 11 pregnancies. Mean age at presentation was 25.45 years and mean gestation when symptoms first developed was 17.09 weeks. Pallor and bleeding manifestations were the most common presenting complaints. Mean Hb, TLC, ANC and platelets were 4.97 g/dl, 2.74 x 10(9)/l, 1.11 x 10(9)/l and 41 x 10(9)/l, respectively. Bone biopsy cellularity ranged from <5 to 25%. Nine out of 11 (81%) pregnancies were successful of which 7 was full term and 2 were premature. Two babies were small for dates. One spontaneous abortion and one intra uterine death (IUD) were observed. Two out of 11 mothers died due to disease after delivery. Two of the 8 surviving mothers, had spontaneous partial response (22%); 4 mothers were asymptomatic after therapy with immunosuppressives given for 6 months and 3 were lost to follow up without response. Specific therapy (cyclosporin) was tried in two mothers antenatally with partial response in one. One child whose mother was given cyclosporin antenatally had jejunal atresia at birth.

CONCLUSION

Pregnancy associated aplastic anemia is a rare association. Spontaneous remission can occur in 25-30% of patients. In the first trimester patients, pregnancy can be terminated while in advanced pregnancy patients can be followed up with stringent supportive care. Cyclosporin may be a safe drug antenatally in such patients. Patients with established aplastic anemia should avoid pregnancy.

摘要

引言

妊娠相关性再生障碍性贫血是一种罕见疾病,其关联尚未得到充分解释。文献中约有80例病例,而我们目前呈现的是迄今为止最大的系列病例,共10例。

结果

10例患者共有11次妊娠。就诊时的平均年龄为25.45岁,症状首次出现时的平均孕周为17.09周。面色苍白和出血表现是最常见的就诊主诉。平均血红蛋白、白细胞计数、中性粒细胞计数和血小板计数分别为4.97g/dl、2.74×10⁹/L、1.11×10⁹/L和41×10⁹/L。骨髓活检细胞比例在<5%至25%之间。11次妊娠中有9次(81%)成功,其中7次为足月分娩,2次为早产。2例婴儿小于孕周。观察到1例自然流产和1例宫内死亡。11例母亲中有2例在分娩后因疾病死亡。8例存活母亲中有2例出现自发部分缓解(22%);4例母亲在接受6个月免疫抑制治疗后无症状,3例失访且无反应。2例母亲在产前尝试了特异性治疗(环孢素),其中1例有部分反应。1例母亲产前接受环孢素治疗的孩子出生时患有空肠闭锁。

结论

妊娠相关性再生障碍性贫血是一种罕见的关联疾病。25% - 30%的患者可出现自发缓解。孕早期患者可终止妊娠,而孕晚期患者可通过严格的支持治疗进行随访。环孢素在这类患者产前可能是一种安全的药物。已确诊再生障碍性贫血的患者应避免妊娠。

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Hematology. 2002 Aug;7(4):233-8. doi: 10.1080/1024533021000024067.
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