A 7-year-old female patient has been initially diagnosed with B-cell precursor acute lymphoblastic leukemia (B-ALL) with hyperdiploidy, which was known to have a favorable prognosis. She underwent combination chemotherapy and achieved initial complete remission. Relapse occurred 6 years following the completion of 2-year chemotherapy, after which combination therapy was repeated, and a second complete remission was achieved. Allogeneic HSCT from a human leukocyte antigen- (HLA-) matched sibling donor was planned when she was 16 years old. Myeloablative preparative chemoradiotherapy, including etoposide at 1800 mg/sqm, CY at 120 mg/kg, and TBI at 12 Gy in six fractions, was performed before HSCT. The patient received an intravenous infusion of bone marrow cells (a total of 3.27 x 108/kg, including 2.3 x 106/kg as CD34+ cells). Short-term methotrexate (15 mg on day +1 and 10 mg on day +3, +6, and +11) and cyclosporine A (3.0 mg/kg) daily from day -1 were used for graft-versus-host disease (GVHD).
On day +17 after HSCT, full-donor chimerism was detected using short tandem repeat analysis. However, she suffered weight gain, ascites, and hepatomegaly and had elevated liver enzymes on day +20 after HSCT. According to the European Society for Blood and Marrow Transplantation (EBMT) criteria, the diagnosis was VOD/SOS, moderate severity. Recombinant human soluble thrombomodulin (rhTM) therapy could not improve liver function.