Abstract
Hepatic veno-occlusive disease (VOD) is a life-threatening complication following hematopoietic stem cell transplantation (HSCT) and associated with a high mortality rate. Therefore, accurate and immediate diagnosis is crucial for implementing appropriate treatment. In our single-center retrospective study, we assessed the accuracy of the Modified Seattle Criteria in children and adolescents undergoing HSCT, and compared them to the diagnostic criteria recently established by the European Society for Blood and Marrow Transplantation (EBMT). Retrospective analysis of medical records of 951 HSCT procedures performed in 850 children and young adults in the years 2001-2015 in the Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation of Wroclaw Medical University Supraregional Center of Pediatric Oncology "Cape of Hope" in Wrocław, Poland. Among the 850 children, 48 were diagnosed with VOD according to the Modified Seattle Criteria (5.05%). Thirteen patients (27%) developed VOD later than within 20 days after transplantation, as required in the diagnostic criteria. Five of the 6 patients who died from VOD were diagnosed with late-onset VOD. Using the categories of symptoms described in the Modified Seattle Criteria, hepatomegaly and weight gain were the most common symptoms in the analyzed cohort (81.25% and 68.75%). Fourteen patients (29%) never demonstrated elevated plasma bilirubin level (>2 mg/dL), as suggested in the Modified Seattle Criteria. Twenty-nine patients (64%) had increased platelet consumption requiring daily transfusions. Only 5 patients with decreased plasma antithrombin III (ATIII) activity level (<80%) on the day of HSCT developed VOD despite supplementation of ATIII. The Modified Seattle Criteria seemed to not meet the special needs of the pediatric population. The new diagnostic criteria proposed by the EBMT appear to be more adequately tailored to the pediatric population and may significantly change the conception of VOD in the future. The surprisingly low incidence of VOD in our cohort may suggest a beneficial role of monitoring and early supplementation of ATIII.
Highlights
Hematopoietic stem cell transplantation (HSCT) has been used as a curative therapy for various kinds of disorders, both malignant and nonmalignant
In our single-center retrospective study, we assessed the accuracy of the Modified Seattle Criteria in children and adolescents undergoing hematopoietic stem cell transplantation (HSCT), and compared them to the diagnostic criteria recently established by the European Society for Blood and Marrow Transplantation (EBMT)
The new diagnostic criteria proposed by the EBMT appear to be more adequately tailored to the pediatric population and may significantly change the conception of veno-occlusive disease (VOD) in the future
Summary
Hematopoietic stem cell transplantation (HSCT) has been used as a curative therapy for various kinds of disorders, both malignant and nonmalignant. Hepatic veno-occlusive disease (VOD), known as sinusoidal obstructive syndrome (SOS), is frequent and may be one of the most severe complications in the early postHSCT period This syndrome is characterized by clinical features like rapid weight gain, ascites, painful hepatomegaly, and jaundice.[1]. Its incidence in the adult population historically has been reported in up to 60%1,4–6 of patients undergoing HSCT, but nowadays it appears to be 13.7%, with values ranging from 0% to 60.2% in different studies.[7] Such great range of results depends on the diagnostic criteria used – the Baltimore or the Seattle Criteria (Table 1).[6,8]. Hepatic veno-occlusive disease (VOD) is a life-threatening complication following hematopoietic stem cell transplantation (HSCT) and associated with a high mortality rate. Accurate and immediate diagnosis is crucial for implementing appropriate treatment
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