Summary:or Baltimore groups. [7][8][9] Thus, in this study, examination was made of changes in hemostatic parameters in children with VOD after BMT. Some parameters appeared appliHepatic veno-occlusive disease (VOD) is a major complication after bone marrow transplantation (BMT). Its cable as markers for VOD and the coagulation-fibrinolysis system was shown to possibly contribute to VOD. prediction, diagnosis and treatment remain unclear. Examination was made of changes in hemostatic parameters in patients with or without VOD after BMT.Patients and methods Twenty-seven children were studied following BMT.
years). The patients plasmin inhibitor/plasmin complex (PIC), antithrombin III (AT-III), protein C, N-terminal propeptide for typewere diagnosed as ALL (n = 11), AML (n = 3), CML (n = 3), severe aplastic anemia (SAA) (n = 3), neuroblas-III procollagen (P-III-P), were measured weekly from pre-BMT to day 28 after BMT. In VOD patients, t-PA toma (NBL) (n = 2), non-Hodgkin's lymphoma (NHL) (n = 1), juvenile chronic myelogenous leukemia (JCML) and PAI-1 significantly increased (P Ͻ0.05) and FBG significantly fell during the post-transplant period (n = 1), severe combined immunodeficiency (SCID) (n = 1), rhabdomyosarcoma (RMS) (n = 1) and peripheral (P Ͻ0.05). Significantly low AT-III and PLG were also noted before VOD (P Ͻ0.05). There were no changes in neuroectodermal tumor (PNET) (n = 1) ( Table 1). Seven patients received autologous marrow grafts and other hemostatic parameters. t-PA, PAI-1 and FBG would thus appear useful markers for the diagnosis of 20, allogeneic marrow grafts (10 HLA-identical siblings, eight HLA-matched unrelated donors, two HLA-mis-VOD, and AT-III and PLG, predictive markers for VOD. The coagulation-fibrinolysis system following matched families). For the conditioning regimen, total body irradiation endothelial cell damage may contribute to the onset of VOD.(TBI) of 12 Gy + thiotepa (TEPA) 600 mg/m 2 + melphalan (LPAM) 140 mg/m 2 was used for 14 patients. Busulphan Keywords: BMT; VOD; hemostatic parameters (BU) 16 mg/kg + cyclophosphamide (CY) 200 mg/kg was administered to five patients, ifosfamide (IFO) 7.5 g/m 2 + LPAM 210 mg/m 2 to four patients, antithymoHepatic veno-occlusive disease (VOD) is a major compliglobulin (ATG) 10 mg/kg + CY 200 mg/kg to two patients, cation of bone marrow transplantation (BMT). Its fre-BU 16 mg/kg + CY 200 mg/kg + TEPA 300 mg/m 2 to one quency varies greatly from 1 to 54%, 1,2 and its high morpatient and BU 16 mg/kg + LPAM 210 mg/m 2 to one tality (30-50%) has not improved. [3][4][5][6] VOD is characterized patient. by hyperbilirubinemia, painful hepatomegaly and ascitesTen patients who had undergone BMT from HLAafter BMT, following chemo-radiotherapy conditioning.matched siblings received cyclosporin A (CsA) alone VOD may possibly arise from injury or disruption of the and/or methotrexate (MTX) as prophylaxis for acute endothelium lining the sinusoids and pores connected to the GVHD, and 10 patients marrow grafts from HLA-matched hepatic venular lumen. End...