儿童溶血危象临床诊断探讨

Clinical observation of children with hemolytic crisis

目的以临床分析为基础,总结儿童急性溶血性贫血(简称急性溶贫)及溶血危象的特征,为"溶血危象"诊断提供临床依据。方法对83例急性溶贫患儿的病因、临床表现、实验室检查、治疗及转归等情况进行回顾性分析。结果随着贫血程度加重,有乏力、苍白、气促、呕吐、酱油色尿、心脏Ⅲ级以上收缩期吹风样杂音临床表现的患儿比例越高;实验室检查中WBC、BUN、AST和LDH升高,以Hb≤70 g/L的患儿改变较为明显,溶血越严重,WBC和LDH升高越明显;血钾、CO2CP、ALT、网织红细胞计数在不同程度急性溶贫的差异无统计学意义(P>0.05)。结论溶血危象诊断要点:(1)确诊急性溶血性贫血;(2)Hb≤70 g/L,同时出现面色苍白、呕吐、酱油色尿、气促、心脏Ⅲ级以上收缩期吹风样杂音和肾功能异常等表现;或Hb≤60 g/L同时出现上述5种以上的表现。具备上述2种者应高度疑诊为溶血危象;(3)如伴有下列表现之一如高热、腹痛、血压下降、意识障碍、惊厥、心力衰竭或急性肾衰竭者即确诊溶血危象;(4)Hb≤30 g/L的极重度溶血性贫血,无论患者的表现如何,均应诊断为溶血危象;(5)外周血WBC>18×109个/L、血清LDH>800 IU/L有助于溶血危象的诊断。

Objective To summarize key points of diagnosis for hemolytic crisis by analyzing clinical data of children with acute hemolytic anemia（AHA）,aimed to supply clinical diagnosis with evidences.Methods Causes,manifestations,laboratory findings,therapy and prognosis of 83 children with AHA were studied retrospectively.Results Clinical features varied with degree of hemolysis,fatigue,severe pallor,anhelation,vomiting,dark urine（tea colored） and blowing murmur of heart were major signs and physical findings in patients with Hb lower than 70 g/L.There were no significant differences for serum potassium,CO2CP,ALT and reticulocyte count among various degrees of hemolysis（P0.05）,but WBC,BUN and LDH increased significantly as deterioration of hemolysis especially for the patients with Hb lower than 70 g/L.Conclusion Key points of diagnosis for hemolytic crisis are suggested：（1） hemolytic anemia is demonstrateded;（2） Hb was lower than 70 g/L and severe pallor,fatigue,vomiting,dark urine,anhelation,heart murmur and renal dysfunction were showed.Hb was lower than 60 g/L and at least five of above manifestations.A diagnosis of hemolytic crisis is highly suspected if a patient presents with condition（1） and（2）;（3） A diagnosis of hemolytic crisis should be made if the patient has one of following characteristics such as high fever,acute abdominal pain,hypotension,unconsciousness,convulsion,heart failure or acute renal failure.（4）Any patient with Hb≤30 g/L should be diagnosis as hemolytic crisis,no matter how is the manifestations.（5） WBC 18 109 and serum LDH 800IU/L are useful for the diagnosis of hemolytic crisis.