Departmental Bulletin Paper 分娩間近に診断され,分娩時大量出血を来した急性全骨髄球性白血病(APL)合併妊娠

久保, 卓郎  ,  井上, 貴至  ,  小野, 哲男  ,  四方, 寛子  ,  喜多, 信幸  ,  三ツ浪, 真紀子  ,  林, 香里  ,  桂, 大輔  ,  天野, 創  ,  辻, 俊一郎  ,  脇ノ上, 史朗  ,  中川, 哲也  ,  石河, 顕子  ,  木村, 文則  ,  高橋, 健太郎  ,  村上, 節  ,  浅井, 愛  ,  南口, 仁志  ,  木藤, 克之

29 ( 1 )  , pp.21 - 26 , 2016-02-05 , 滋賀医科大学雑誌編集委員会
Acute promyelocytic leukemia(APL)occupies approximately about 10~15% of acute myeloblastic leukemia. After the introduction of all-trans retinoic acid(ATRA)therapy, the prognosis of APL has been dramatically improved. However, APL still remains life-threatening, because disseminated intravascular coagulation(DIC)occurs in large portions of patients with this disease. Here we report a case of the pregnant woman who was diagnosed with APL in the third trimester, and had no time to receive ATRA therapy before delivery. We managed this patient, although she suffered from massive post-partum hemorrhage due to severe hypo-fibrinogenaemia.A pregnant woman noticed purpuras on the trunk and upper extremities after the gestational age of 30 weeks. At the gestational age of 37 weeks, she had severe nasal bleeding continuing for an hour, and the blood test revealed pancytopenia. When she was referred to our hospital, DIC was accompanied with pancytopenia. Bone marrow aspiration was performed to obtain diagnosis, and the result showed 88% abnormal promyelocytes with fine heavy granules and fagotts. The findings were compatible with APL. Immediately after the admission, spontaneous labor began and she delivered 2978 g of a male infant. After the delivery of the placenta and the suture of the vaginal laceration, massive post-partum hemorrhage continued and the vaginal wall hematoma developed. Severe hypo-fibrinogenaemia persisted despite FFP replacement. Two hours after the delivery, the amounts of bleeding reached to 3,200 g. With the use of 2 g of fibrinogen concentrate, we conducted suture under general anesthesia, and finally the bleeding decreased. Then, the patient was treated by ATRA therapy and chemo-therapy. Three months later she had gained complete remission from APL. This case shows that fibrinogen concentrate is useful to control huge postpartum hemorrhage in APL patients with hypo-fibrinogenaemia due to DIC.

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