@article{oai:hama-med.repo.nii.ac.jp:00003377,
 author = {熊澤, 理紗 and 水野, 薫子 and 南波, 美沙 and 米澤, 真澄},
 issue = {1},
 journal = {静岡産科婦人科学会雑誌},
 month = {Mar},
 note = {May-Hegglin異常は血小板減少症、巨大血小板、および顆粒球封入体を特徴とする稀な常染色体優性遺伝疾患である1)。今回我々は、May-Hegglin異常合併妊娠の一例を経験した。患者は29歳の初産婦で、4歳時より特発性血小板減少性紫斑病（idiopathic thrombocytopenic purpura; ITP）として経過観察されていた。自然妊娠し、13週2日、当科初診となった。初診時の血小板数は4.0×104/μLであった。当院血液内科では、家族歴に実父の血小板減少があることからMay-Hegglin異常を疑い、塗抹標本で巨大血小板と顆粒球封入体を認めたためMay-Hegglin異常と診断した。血小板数は20週の時点で2.6×104/μLまで減少したが、以降は上昇し36週時には12.4×104/μLであった。38週2日、健診で羊水過少のためterminationの方針とし、38週3日、誘発頭位分娩に至った。分娩時出血量は275gであった。児にも血小板減少 (6.4×104/μL)、巨大血小板、顆粒球封入体を認め、May-Hegglin異常と診断された。May-Hegglin異常合併妊娠の管理では、①母児の出血リスク評価、出血対策、②児のAlport症状（腎炎、難聴、白内障）合併の可能性を念頭に置くこと、③妊産婦、家族への情報提供 の3点が重要である。, May-Hegglin anomaly is a rare autosomal dominant disorder characterized by thrombocytopenia, giant platelets, and granulocyte inclusions. We experienced a patient whose pregnancy was complicated by May-Hegglin anomaly. The patient was a primipara 29 years of age with idiopathic thrombocytopenic purpura (ITP) which had been diagnosed when she was 4 years old. She conceived naturally and visited our department at 13 weeks and 2 days of pregnancy. At the first visit, the platelet count was 4.0×104/μL. At the hematology department of our hospital, May-Hegglin anomaly was suspected based on family history; her father also had thrombocytopenia. Giant platelets and granulocyte inclusions were identified by smear preparation and May-Hegglin anomaly was thus diagnosed. The platelet count decreased to 2.6×104/μL at 20 weeks but then rose thereafter to 12.4×104/μL at 36 weeks. At 38 weeks and 2 days, examination revealed oligohydramnios, and we thus decided to induce labor. The patient delivered in cephalic presentation after induction of labor at 38 weeks and 3 days. The intrapartum hemorrhage was 275 g. Thrombocytopenia (6.4×104/μL), giant platelets, and granulocyte inclusions were observed in the child, confirming a diagnosis of May-Hegglin anomaly. In managing women whose pregnancy is complicated by May-Hegglin anomaly, the following three issues are important: (1) Evaluation of hemorrhage risk and measures against hemorrhage in both the mother and the child, (2) Paying close attention to whether the child’s course is complicated by Alport symptoms (nephritis, hearing impairment, and cataract), and (3) Providing accurate and useful information to expectant mothers and their families.},
 pages = {37--43},
 title = {May-Hegglin異常合併妊娠の一例},
 volume = {8},
 year = {2019}
}