日本新生児成育医学会雑誌　第30巻　第1号　27～32頁（2018年）

日本新生児成育医学会雑誌　30(1)：27-32；2018　

受付日：平成28．12．02

受理日：平成29．05．15

ステロイド抵抗性　新生児Kasabach-Merritt 現象に対し短期間のビンクリスチン併用で治癒した1 例

Successful Treatment of Steroid-Resistant Kasabach-Merritt Phenomenon in a Neonate Undergoing Short-term Vincristine Administration

＊1長野県立こども病院　新生児科，＊2長野県立こども病院　血液腫瘍科，＊3信州大学　医学部　新生児学講座

＊1Division of Neonatology, Nagano Children’s Hospital，＊2Department of Hematology and Oncology, Nagano Children’s Hospital，＊3Division of Neonatology, Shinshu University School of Medicine

氏家紘平＊1・関　聡子＊1・能見恭子＊1・福嶋ゆう＊1・中村千鶴子＊1・山口由美＊1・亀井良哉＊1・伊藤有香子＊1・溝上雅恵＊1・小田　新＊1・小久保雅代＊1・廣間武彦＊1・坂下一夫＊2・中村友彦＊1＊3

Kohei UJIIE＊1，Satoko SEKI＊1，Kyoko NOMI＊1，Yu FUKUSHIMA＊1，Chizuko NAKAMURA＊1， Yumi YAMAGUCHI＊1，Yoshiya KAMEI＊1，Yukako ITO＊1，Masae MIZOGAMI＊1，Arata ODA＊1， Masayo KOKUBO＊1，Takehiko HIROMA＊1，Kazuo SAKASHITA＊2，Tomohiko NAKAMURA＊1＊3

Key Words：neonate，Kasabach-Merritt phenomenon，vincristine

　症例は在胎34 週5 日の胎児エコーで頭頸部巨大血管腫，胎児水腫を指摘され，在胎35 週0 日に胎児機能不全の適応で緊急帝王切開となった。出生直後から重篤な血小板減少と凝固障害を合併しておりKasabach-Merritt 現象（Kasabach-Merritt phenomenon：KMP）と診断した。ステロイドは無効で日齢5 からビンクリスチン（vincristine： VCR）併用を開始し，血小板減少と凝固障害が改善した。しかし原因不明の胆汁鬱滞とVCR による骨髄抑制により 3 クール目のVCR 投与を中断したところKMP が再燃したためVCR 投与を再開した。血小板数は正常化したが，再び胆汁鬱滞の増悪と骨髄抑制が出現したため計5 クールのVCR 投与で終了した。その後KMP は再燃せず腫瘍は退縮した。KMP に対するVCR 治療は20 週以上の長期間行われることが多いが，本症例の経過から血小板数の正常化と腫瘍の縮小を指標にすればVCR 治療期間を短縮できる可能性がある。

　We describe a case of a male infant who presented with a large cervicofacial hemangioma associated with Kasabach- Merritt phenomenon who was successfully treated with short-term vincristine administration. A large cervicofacial hemangioma and fetal hydrops were identified using fetal ultrasonography and magnetic resonance imaging（MRI）at the gestational age of 34.5 weeks, and the mother was transferred to our hospital. An emergency caesarean section was performed at the gestational age of 35.0 weeks since the fetal status was not considered stable. The Apgar score was 3 at 1 min and 7 at 5 min. Since complications of serious coagulopathy and severe thrombocytopenia were seen postpartum, Kasabach-Merritt phenomenon was diagnosed. Prednisolone was administered but proved ineffective. As such, vincristine was administered on days 5 and 12（0.025mg/kg/dose, half-doses of the usual dose due to observed liver dysfunction and cholestatic jaundice）. However, the infant experienced neutropenia and worsening cholestatic jaundice as side effects of vincristine. We therefore decided to discontinue vincristine administration. We resumed administration of half-dose vincristine from day 24 since a relapse in the Kasabach-Merritt phenomenon was observed. Kasabach-Merritt phenomenon typically resolves after 5 administrations of vincristine. Kasabach-Merritt phenomenon can be successfully treated using short-term vincristine if attention is paid to observed side effects.