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High-dose methylprednisolone for the treatment of sinusoidal obstruction syndrome in adults

Bone Marrow Transplantation volume 53, pages 923–925 (2018)Cite this article

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Sinusoidal obstruction syndrome or hepatic veno-occlusive disease (SOS/VOD) occurs either as early or late onset (i. e. within 21 days or beyond day 21, respectively) complication after hematopoietic stem cell transplantation (HSCT) and is characterized by hyperbilirubinemia, ascites or weight gain, right upper quadrant pain of liver origin, and tender hepatomegaly [1]. SOS/VOD may be a self-limited disease. However, in severe SOS/VOD cases >80% of patients die due to multiorgan failure (MOF) as well as other complications [2]. Defibrotide is the only therapeutic agent approved for the treatment of severe SOS. High-dose methylprednisolone (HDMP) showed encouraging response rates in patients with SOS, but defibrotide and some other concomitant medications (alteplase, streptokinase, PGE1, etc.) have also been used in a number of reports [3, 4] making the HDMP clinical value less clear. Here we describe our results of treating adult SOS/VOD after HSCT with HDMP and without the use of defibrotide or any other concomitant therapy.

We retrospectively reviewed all patients who received SCT at our institution during a 10-year period (2007–2016). During this period, defibrotide was periodically not available and HDMP was the treatment of choice. We identified 11 patients who fulfilled the recently published EBMT diagnostic criteria for SOS [1] and were treated with HDMP, without the concomitant use of defibrotide at any time point during their disease course. No other agents such as TPA in addition to HDPM were used due to possible risk of treatment-related toxicity. Patient characteristics are shown in Table 1. SOS/VOD was diagnosed on the median 22 (7–81) days after HSCT. The median bilirubin at diagnosis of SOS/VOD was 70.7 (11.1–137) µmol/L for the entire study population. One patient had normal bilirubin value but similarly to the remaining patients fulfilled the EBMT criteria for SOS/VOD and displayed signs such as ascites and weight gain, right upper quadrant pain of liver origin, and tender hepatomegaly. A response was defined as a decrease in total serum bilirubin concentration by 50% from the baseline and resolution of all SOS/VOD related clinical symptoms within 1 month from the start of HDMP. For patients with normal serum bilirubin values, a response was defined as regression of symptoms associated with SOS/VOD. The patients had no signs of aGvHD and hepatitis of infectious origin was ruled out at the time of SOS/VOD diagnosis.

Table 1 Patient characteristics
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Authors and Affiliations

  1. Faculty of Medicine, Vilnius University, Vilnius, Lithuania

    Mantas Vaisvilas, Laimonas Griskevicius & Valdas Peceliunas

  2. Hematology, Oncology and Transfusion Medicine Center, Vilnius University Hospital Santaros Klinikos, Vilnius, Lithuania

    Skirmante Buseckaite, Orinta Mickeviciute, Rita Cekauskiene, Laimonas Griskevicius & Valdas Peceliunas

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  1. Mantas Vaisvilas
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  2. Skirmante Buseckaite
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Correspondence to Mantas Vaisvilas.

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Vaisvilas, M., Buseckaite, S., Mickeviciute, O. et al. High-dose methylprednisolone for the treatment of sinusoidal obstruction syndrome in adults. Bone Marrow Transplant 53, 923–925 (2018). https://doi.org/10.1038/s41409-018-0087-7

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