| Objective:To summarize the clinical features of sinusoidal obstruction syndrome (SOS), improve the understanding of this disease and reduce the chances of misdiagnosis or missed diagnosis.Methods:A retrospective study was performed on the clinical data of 22 children with SOS including clinical manifestations, laboratory results, imaging findings, treatments and prognosis.Results:(1) Among 22 cases,15 were male (68.2%),7 were female (31.8%), the sex ratio as 2.14 to 1.The age of onset among 22 cases were 3 years 2 months to 12 years 2 months,6.7 years on average.13 cases were preschool,5 cases were school-age and 4 cases were adolescent.(2) The primary disease of 18 cases was acute lymphoblastic leukemia (ALL),13 of them used 6-Tioguanine(6-TG),5 of them used 6-Mercaptopurine(6-MP). Other 4 cases were thalassemia major, who underwent hematopoietic stem cell transplantation and also used busulfan/cyclophosphamide (BU/CY) as chemotherapy.(3) Clinical manifestations mainly included abdominal distention and loss of appetite (6 cases), hepatomegaly with tenderness on palpation (14cases), and weight gain(14 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 10 cases. Out of the 12 patients who underwent serum D-dimer test,5 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 14 cases. Ascites of 2 cases, with elevated level of total protein and serum-ascitic albumin gradient (SAAG),1 case showed elevated level of ascites CA125. Serum CA125 elevated in 1 case.(4) There were 12 cases in acute stage(54.5%),4 cases were sub-acute (18.2%),6 cases were chronic(27.3%). Five cases of SOS were mild in clinical grading(22.7%),2 cases were moderate(9.1%),15 cases were severe(68.2%). The severity of SOS was unrelated with gender, primary disease and clinical stages (P>0.05). Severity showed relation with age, patients of preschool age might present more severe manifestation than school-age and adolescence. (P <0.05). ALL patients with the history of using 6-TG, might present with more severe clinical manifestation than those with the history of using 6-MP (P <0.05).(5) Ten patients were subjected to color Doppler ultrasound examination, 5 cases were subjected to CT scan. 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.(6) Patients were mainly treated with liver protection, improving microcirculation, anticoagulant and diuretics; the prognosis was good in all patients.Conclusions:(1) SOS may occur in patients who received chemotherapy for leukemia and hematopoietic stem cell transplantation, especially among patients who underwent regimen of 6-TG or BU/CY.(2) Clinical manifestations of SOS mainly included abdominal distention, loss of appetite, hepatomegaly with tenderness on palpation and weight gain, which showed no specificity.(3) Patchy liver enhancement, hepatic veins narrowing or unclear in color Doppler ultrasound examination or CT scan highly demonstrated the diagnosis of SOS.(4) Characterized by acute onset and severe clinical condition of SOS, early diagnosis and treatment should be emphasized. Treatments were basically symptomatic and supportive therapies. |
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