Gill Martin
The Heart Centre for Children, The Sydney Children's Hospitals Network, Sydney, Australia.
J Extra Corpor Technol. 2019 Jun;51(2):83-87.
Thrombocytopenia is a life-threatening condition, the severity of which is exacerbated further if the patient requires anticoagulation and is refractory to platelet transfusion. This is the first report of an infant undergoing extracorporeal support with immune-mediated platelet refractoriness. A 19-month-old girl, with a complex cardiac history, required extracorporeal support because of deterioration 8 days post-cardiac surgery. The child suffered from ongoing thrombocytopenia, unresponsive to multiple platelet transfusions. An incremental rise in the platelet count was achieved following transfusion of human leukocyte antigen-matched platelets, although this was unsuccessful with subsequent transfusions of matched platelets. Following 7 days on extracorporeal membrane oxygenation (ECMO), without cardiac improvement and likely poor prognosis, treatment was withdrawn and the patient died. The management of immune-mediated platelet refractoriness, in an anticoagulated patient on ECMO, requires early diagnosis and timely intervention to achieve a good outcome for the child. An understanding of the condition and a multidisciplinary approach to its treatment will assist in effective direction of medical therapy.
血小板减少症是一种危及生命的疾病,如果患者需要抗凝治疗且对血小板输注无效,其严重程度会进一步加剧。这是首例接受体外支持治疗且伴有免疫介导的血小板输注无效的婴儿报告。一名19个月大、有复杂心脏病史的女孩,因心脏手术后8天病情恶化而需要体外支持。该患儿持续存在血小板减少症,对多次血小板输注均无反应。输注人白细胞抗原匹配的血小板后,血小板计数呈渐进性上升,尽管随后输注匹配的血小板未成功。在接受体外膜肺氧合(ECMO)治疗7天后,心脏状况未改善且预后可能不佳,于是停止治疗,患者死亡。对于接受ECMO治疗的抗凝患者,免疫介导的血小板输注无效的管理需要早期诊断和及时干预,以让患儿获得良好预后。了解该疾病并采用多学科方法进行治疗将有助于有效指导药物治疗。