Bontant Thomas, Le Garrec Sophie, Avran David, Dauger Stephane
PICU, Hopital Robert Debré, AP-HP and Paris Diderot, Paris 7 University, Paris, France.
BMJ Case Rep. 2014 Aug 12;2014:bcr2014204706. doi: 10.1136/bcr-2014-204706.
A 5-year-old boy from the Congo, was admitted for hyperleucocytic acute lymphoblastic leukaemia, with a high risk of tumour lysis syndrome (TLS). He had splenomegaly and mediastinal lymphadenopathy on chest X-ray. We started steroids and hyperhydration with rasburicase to prevent TLS. Respiratory failure with mediastinal enlargement developed rapidly. A few hours after intensive care unit (ICU) admission, he was started on mechanical ventilation. Chemotherapy was started immediately given the strong suspicion of mediastinal compression. Low oxygen saturation with high partial arterial oxygen pressure persisted. Blood tests confirmed 20% methaemoglobinaemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Allopurinol was substituted for rasburicase. The methaemoglobinaemia disappeared rapidly and he was discharged from the ICU after 72 h. In case of rasburicase use, a close clinical monitoring is mandatory, especially in populations where G6PD deficiency is highly prevalent. Methaemoglobinaemia must be suspected in case of low oxygen saturation when all other potential causes have been ruled out.
一名来自刚果的5岁男孩因高白细胞性急性淋巴细胞白血病入院,有发生肿瘤溶解综合征(TLS)的高风险。他有脾肿大,胸部X线显示纵隔淋巴结肿大。我们开始使用类固醇和用拉布立酶进行水化以预防TLS。迅速出现了伴有纵隔增宽的呼吸衰竭。入住重症监护病房(ICU)数小时后,他开始接受机械通气。鉴于高度怀疑纵隔受压,立即开始化疗。低氧饱和度伴高动脉血氧分压持续存在。血液检查证实有20%的高铁血红蛋白血症和葡萄糖-6-磷酸脱氢酶(G6PD)缺乏。用别嘌醇替代了拉布立酶。高铁血红蛋白血症迅速消失,72小时后他从ICU出院。在使用拉布立酶的情况下,必须进行密切的临床监测,尤其是在G6PD缺乏高度流行的人群中。当排除了所有其他潜在原因而出现低氧饱和度时,必须怀疑有高铁血红蛋白血症。