肝窦阻塞综合征12例临床分析

Clinical features of sinusoidal obstruction syndrome:report of 12 cases

摘要:

目的 总结肝窦阻塞综合征(SOS)的临床特点,以提高对该病的认识。方法 回顾性分析12 例SOS患儿的临床表现、实验室检查、影像学表现、治疗及预后等情况。结果 12 例患儿中,8 例患儿原发病为白血病,4 例为造血干细胞移植患儿。临床表现主要有腹胀(4例)、肝肿大伴触痛(9例)、脾大(6例)、体重增加(10例)。12 例患儿均有不同程度的肝损害;凝血功能检测中活化部分凝血活酶时间延长7 例;7 例患儿行血D-二聚体检查,其中4 例升高。血常规检测中,4 例白细胞、粒细胞均降低;9 例有不同程度的血小板降低。8 例患儿行彩色多普勒超声检查,可见弥漫性肝肿大、实质非均质改变,肝静脉变细、显示不清或胸腹水、脾大。2 例行肝活检,示肝窦内细胞浸润或肝窦扩张。治疗以对症支持为主,均预后良好。结论 白血病及干细胞移植患儿治疗后如果发生肝肿大、体重突然增加、肝功能异常、凝血功能异常等表现,需警惕SOS 的发生。

Abstract:

Objective To summarize the clinical features of sinusoidal obstruction syndrome (SOS) and to improve the understanding of this disease.Methods A retrospective study was performed on the clinical data of 12 children with SOS including clinical manifestations, laboratory results, imaging findings, treatment, and prognosis.Results Of the 12 cases, 8 were secondary to acute lymphoblastic leukemia, and 4 had undergone hematopoietic stem cell transplantation. Manifestations mainly included abdominal distention (4 cases), hepatomegaly with tenderness (9 cases), splenomegaly (6 cases), and weight increase (10 cases). Biochemical tests revealed varying degrees of liver damage in all cases. In the coagulation function test, the activated partial thromboplastin time (APTT) was prolonged in 7 cases. Out of the 7 patients who underwent serum D-dimer test, 4 showed elevated serum level of D-dimer. In routine blood tests, 4 cases showed decreases in both white blood cells and neutrophils. In addition, varying degrees of thrombocytopenia were observed in 9 cases. Eight patients were subjected to color Doppler ultrasound examination, and diffuse hepatomegaly, inhomogeneous liver parenchyma, unclear or thinner hepatic veins, hydrothorax/ascites, or splenomegaly was observed. Sinusoidal dilatation or hepatic cell infiltration was observed in 2 patients who underwent liver biopsy. Treatments were basically symptomatic and supportive therapies, and the prognosis was good in all patients.Conclusions SOS should be considered in children who present with hepatomegaly, sudden weight gain, liver dysfunction and coagulation dysfunction after they have received chemotherapy for leukemia and hematopoietic stem cell transplantation.