Veno-occlusive disease/sinusoidal obstruction syndrome after haematopoietic stem cell transplantation: Middle East/North Africa regional consensus on prevention, diagnosis and management

Abstract

Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) of the liver is a serious, early complication of haematopoietic stem cell transplantation (HSCT), severe and very severe forms of which are associated with a high mortality rate. A wide variety of patient, disease and treatment-related risk factors for VOD/SOS have been identified. Several bodies have published recommendations for the diagnosis, prevention and management of VOD/SOS following HSCT. A group of regional experts have developed a consensus statement on the diagnosis, prevention and management of VOD/SOS in the Middle East and North Africa region to help in the management of HSCT patients in the region. Risk factors of particular relevance in the region include iron overload in thalassaemia patients, some hereditary metabolic disorders due to consanguinity and infection with hepatitis virus B or C. Recommendations include diagnosis of VOD/SOS based on established clinical criteria, prophylaxis with defibrotide and/or ursodeoxycholic acid in patients at increased risk of VOD/SOS, and treatment with defibrotide for patients with severe/very severe VOD/SOS (and, if clinically indicated, in those with moderate or rapidly progressing VOD/SOS, as per the new European Society for Blood and Marrow Transplantation classification).

Sinusoidal obstruction syndrome: A systematic review of etiologies, clinical symptoms, and magnetic resonance imaging features

“…However, both of the patients developed severe abdominal pain, hepatomegaly, and ascites approximately 2 wk after receiving HSCT, and the patients who received allogeneic transplantation progressed rapidly to upper gastrointestinal bleeding. Therefore, we believed that the occurrence of these 2 SOS cases is consistent with that mentioned by Al Jefri et al[4].…”

“…Some risk factors for SOS related to HSCT have been identified, including existing liver disease (chronic hepatitis, liver fibrosis, and cirrhosis), prior history of liver radiant examination, and the effects of some drugs used in the process of conditioning[12]. In addition, Al Jefri et al[4] pointed out that if a patient was too young or too old, or accepted allogeneic transplantation, the possibility of morbidity was greatly increased. In our included literature, van den Bosch et al[13] reported 2 patients who received HSCT due to a history of leukemia.…”

“…Although liver biopsy is the gold standard for the diagnosis of SOS, leukopenia and poor liver function resulting from hematological diseases or advanced tumors make this operation difficult. The Baltimore criteria, the modified Seattle criteria, and the European Society for Blood and Marrow Transplantation criteria are the three main criteria for diagnosing SOS[4,5]. However, these criteria are usually appropriated for SOS secondary to haemopoietic stem cell transplantation (HSCT), including a little of clinical information but not involving imaging findings.…”

Tandem high‐dose chemotherapy with autologous stem cell rescue for stage M high‐risk neuroblastoma: Experience using melphalan/etoposide/carboplatin and busulfan/melphalan regimens

“…Repeated HDCT would cause more complications than single HDCTs and BU use in the second HDCT. 19,20 Adequate prophylaxis for SOS was conducted in our cohort, and consequently, SOS was not observed. Furthermore, pharmacokinetic analysis could individualize BU dosage, which would also be useful to reduce the risk of SOS.…”

“…The BU dosage was determined by the patient's BW per the manufacturer's directions as follows: dosages of 1.0, 1.2, 1.1, 0.95, and Oral administration of ursodeoxycholic acid was added to HDCT with BuMel to reduce the risk of SOS by repeated doses of HDCT and the use of BU. 19,20 Granulocyte colony-stimulating factor was administered intravenously beginning 5 days after the stem cell rescue until the white blood cell count was >1000/µL. A median number of 1.9 × 10 6 and 1.1 × 10 6 CD34-positive cells/kg obtained from apheresis of peripheral blood were administered for the first and second stem cell rescues, respectively.…”