Yang Yi, Guo Zhiheng, Wang Zhenpeng, Luo Lili, Chen Ying
Center of Reproductive Medicine and Center of Prenatal Diagnosis.
Department of Obstetrics.
Medicine (Baltimore). 2020 Jul 10;99(28):e21198. doi: 10.1097/MD.0000000000021198.
Kasabach-Merritt Syndrome (KMS) is an extremely rare disease in adults, which lead to consumptive coagulopathy characterized by severe hypofibrinogenemia and thrombocytopenia. PATIENT CONCERNS:: a 25-year-old Chinese pregnant women complicated by preeclampsia and KMS presented with refractory postpartum hemorrhage and incision bleeding after cesarean section.
The diagnosis of KMS was made based on clinical manifestation of Kaposiform Hemangioendothelioma, severe hypofibrinogenemia and thrombocytopenia.
After a poor response to massive blood products transfusion for 1 week, corticosteroid treatment was initiated for 3 days.
The patient reached a normal platelet count and a mild anemia within 4 weeks. Two months later, all laboratory values had returned to normal, and the incision was healing well.
Pregnancy complicated by preeclampsia and surgery may have contributions for the development of Kasabach-Merritt syndrome. Corticosteroid is indicated in the episode of acute Kasabach-Merritt syndrome after the failure of massive blood transfusion.
卡萨巴赫-梅里特综合征(KMS)在成人中极为罕见,可导致以严重纤维蛋白原血症和血小板减少为特征的消耗性凝血病。患者情况:一名25岁的中国孕妇并发先兆子痫和KMS,剖宫产术后出现难治性产后出血和切口出血。
根据卡波西样血管内皮瘤的临床表现、严重纤维蛋白原血症和血小板减少症做出KMS的诊断。
在大量输注血液制品1周效果不佳后,开始使用皮质类固醇治疗3天。
患者在4周内血小板计数恢复正常,出现轻度贫血。两个月后,所有实验室值均恢复正常,切口愈合良好。
并发先兆子痫和手术的妊娠可能对卡萨巴赫-梅里特综合征的发生有影响。在大量输血失败后的急性卡萨巴赫-梅里特综合征发作时,皮质类固醇是适用的。