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目的了解急性淋巴细胞白血病(ALL)经环磷酰胺(CTX)、阿糖胞苷(Ara-c)、6-硫鸟嘌呤(6-TG)(CAT方案)化疗后发生肝静脉闭塞病(VOD)的临床和预后特征,探讨VOD的发病机制、预防措施及治疗方法。方法对2008年4月-2009年4月本科经CAT方案化疗后发生VOD的4例ALL患儿的临床资料进行回顾性分析。4例患儿在CAT方案化疗后均出现腹痛、腹胀,并伴黄疸、腹水及肝大,实验室检查血常规、肝功能、出凝血指标均异常,且血小板输注无效。予对症支持、保肝及小剂量肝素治疗。结果3例患儿经积极治疗后血常规、肝功能、出凝血指标、彩色多普勒检查腹水均较治疗前明显改善,临床症状缓解。1例患儿病情进展迅速,抢救无效死亡。结论ALL患儿发生VOD可能与CAT方案化疗药物联合不良反应有关,血常规变化尤其是血小板下降,常早于VOD的临床症状,早期诊断和积极干预治疗对改善预后至关重要,小剂量肝素在VOD治疗中起重要作用。
Objective To investigate the incidence of hepatic veno-occlusive disease (VOD) in patients with acute lymphoblastic leukemia (ALL) treated with CTX, Ara-c and 6-thiguanine (CAT) ) Of the clinical and prognostic characteristics, explore the pathogenesis of VOD, preventive measures and treatment. Methods The clinical data of 4 children with ALL who underwent VOD after CAT regimen chemotherapy from April 2008 to April 2009 were analyzed retrospectively. All the 4 patients had abdominal pain, abdominal distension, jaundice, ascites and hepatomegaly after the chemotherapy of CAT. The blood tests, liver function and coagulation indexes in laboratory were abnormal, and the platelet transfusion was ineffective. To symptomatic support, liver protection and low-dose heparin treatment. Results After 3 cases of active treatment, blood routine, hepatic function, coagulation index and color Doppler echocardiography were significantly improved compared with those before treatment, and clinical symptoms were relieved. One case of children progressed rapidly, rescue died. Conclusions VOD in children with ALL may be associated with adverse reactions of CAT chemotherapy. The blood routine changes, especially thrombocytopenia, are often earlier than the clinical symptoms of VOD. Early diagnosis and active intervention are important for improving prognosis. Low dose heparin VOD treatment plays an important role.