骨髄異形成症候群合併妊娠:─症例報告と文献による51例の検討─  [in Japanese] Pregnancy complicated with myelodysplastic syndrome:-report of a case and review of the literature-  [in Japanese]

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Abstract

骨髄異形成症候群 myelodysplastic syndorme (MDS)は,造血幹細胞の異常クローン増殖により汎血球減少と無効造血をきたし,進行性・治療抵抗性のため予後不良な疾患である.MDSは妊娠中に増悪することもあり,子宮内胎児発育遅延や胎児機能不全等の合併症の発生リスクが高くなるとされている.今回われわれはMDS合併妊娠で,分娩周辺期の血小板輸血により母児を安全に管理し得た.本邦での過去の症例51例と合わせて報告する.症例は29歳,初産婦.2年前よりMDSに対し血液内科にて経過観察中であった.白血球や赤血球の著明な減少は認めなかったが,血小板減少を認めていた.近医にて妊娠判明し,妊娠12週で当科紹介受診となった.徐々に血小板が減少したため,妊娠29週より入院管理とした.妊娠中出血傾向を認めなかったため,血小板輸血は行わなかった.妊娠37週1日に選択的帝王切開術施行したが,術直前および術直後に血小板輸血を行い,術中および術後に出血傾向はなかった.母児ともに経過観察中であるが,MDSの増悪や新生児への影響は現在のところ認められていない.これまでのMDS合併妊娠の報告では,治療抵抗性の貧血を認め,妊娠中に初めてMDSと診断された症例が53%(28/52)であった.合併症として妊娠高血圧症候群や胎児機能不全が多い傾向にあった.妊娠時や分娩時に出血傾向はそれほど認められなかったが,ほとんどの症例で血小板輸血を行った.十分な血液製剤が確保できるのであれば,産科的禁忌がない限り経腟分娩は可能と考えるが,高次医療施設での妊娠・分娩管理が必要と考える.妊娠中に治療抵抗性の貧血や血球減少を認めた場合は,血液内科医と連携し速やかに骨髄穿刺にて診断を行い,MDSと診断されれば慎重な妊娠・管理が必要である.〔産婦の進歩63(2):112-120,2011(平成23年5月)〕

The myelodysplastic syndromes (MDS) are a group of conditions that are progressive and refractory to treatment, characterised by proliferation of abnormal clones of myeloid stem cells resulting in pancytopenia and ineffective hematopoiesis. Deterioration is often seen during pregnancy with MDS, and the risk of complications, such as intrauterine growth restriction and fetal distress, is high. In this report, we present a case of pregnancy complicated by MDS, in which we were able to manage the mother body and the fetus safely by the platelet transfusion of the period around delivery. The patient was a 29 year old primigravida. She had been under treatment for 2 years for MDS. Thrombocytopenia was present, with no marked decrease in red or white cell counts. Pregnancy was confirmed at 12 weeks' gestation. Her platelet count gradually declined, requring admission at 29 weeks' gestation. Since no hemorrhagic tendency was detected during the pregnancy, no platelet transfusions were given. An elective Caesarean section was performed at 37 weeks and 1 day gestation, with pre- and postoperative platelet transfusions, and good control of bleeding during and after the procedure. Both mother and child remained well, with no signs to date of deterioration of the mother's MDS, or adverse effects on the child. A review of the literature regarding pregnancies complicated by MDS revealed that 53% of cases (28/52) were first diagnosed with MDS during the pregnancy. Complications with hypertension during pregnancy and fetal distress were common. Hemorrhagic tendencies during the pregnancy or at the time of delivery were not common, although almost all cases required platelet transfusions. If adequate supplies of blood products are available, vaginal birth is possible unless there are no other obstetric contraindications, although the pregnancy and delivery should be managed properly. If refractory anemia or cytopenia is detected in a pregnant woman, a hematological consultation should be done as soon as possible. We propose that if MDS is diagnosed, appropriate management will be required for the pregnancy and delivery. [Adv Obstet Gynecol, 63 (2) : 112-120, 2011 (H23.5)]

Journal

  • ADVANCES IN OBSTETRICS AND GYNECOLOGY

    ADVANCES IN OBSTETRICS AND GYNECOLOGY 63(2), 112-120, 2011

    THE OBSTETRICAL GYNECOLOGICAL SOCIETY OF KINKI DISTRICT JAPAN

Codes

  • NII Article ID (NAID)
    130000904752
  • NII NACSIS-CAT ID (NCID)
    AN00099490
  • Text Lang
    JPN
  • ISSN
    0370-8446
  • NDL Article ID
    11101644
  • NDL Source Classification
    ZS38(科学技術--医学--産婦人科学)
  • NDL Call No.
    Z19-310
  • Data Source
    NDL  J-STAGE 
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