1/5. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer.hepatic veno-occlusive disease (VOD) is a well-known complication of chemotherapy in wilms tumor patients, particularly young children. Although this complication resolves uneventfully in most patients, fatal cases have been reported. Severe VOD after transplantation has a high mortality rate ranging from 45% to 98%. New hemostatic therapeutic strategies have significantly improved the prognosis of VOD. Chemotherapy-related VOD in wilms tumor usually has a good prognosis. We describe two patients with wilms tumor and one with acute lymphoblastic leukemia, who developed severe veno-occlusive disease of the liver according to the baltimore criteria while undergoing chemotherapy; the symptoms were hepatomegaly, ascites, hyperbilirubinemia, weight gain and, in one patient, short-term lethargy. Elevated LDH levels of 872 to 12,000 U/l were observed in our patients. All patients had thrombocytopenia between 29,000 and 40,000/microl and decreased antithrombin (AT) and protein c levels; two patients had gastrointestinal bleeding. All patients developed a coagulopathy because of severe hepatic dysfunction. Two patients received low-dose heparin at the onset of VOD. The thrombolytic therapy was rapidly changed to AT supplementation (20-80 IU/kg bw 2x per day) without heparin when thrombocytes were very low or gastrointestinal bleeding occurred. Resolution of VOD was observed in all patients receiving AT alone. The chemotherapy was discontinued in a patient with accidental actinomycin D overdosage in view of the severity of symptoms. The remaining two patients received chemotherapy according to the therapy protocol after restitution. All patients survived without sequelae with a median follow-up of 28 months (range 8-48 months). CONCLUSION: hepatic veno-occlusive disease is a rare but increasingly recognized complication in pediatric cancer patients receiving conventional chemotherapy. AT supplementation constitutes a good alternative treatment of severe VOD in comparison with other thrombolytic therapies, particularly in patients at high risk of bleeding.- - - - - - - - - - ranking = 1keywords = alternative (Clic here for more details about this article) |
2/5. Sonographic diagnosis of venocclusive disease of the liver and danazol therapy for autoimmune thrombocytopenia in an autologous marrow transplant patient.A 16-Year-old boy with lymphoblastic lymphoma underwent an autologous bone marrow transplantation (ABMT) after conditioning with high-dose busulfan and cyclophosphamide. On day 39 post-transplant, right upper quadrant pain occurred with an increase in the size of the liver. liver function tests showed a subsequent deterioration. Ultrasonographic studies of the abdomen disclosed hepatosplenomegaly, ascites, thickening of the gall bladder wall and a failure to visualize the major hepatic veins. The venocclusive disease of the liver (VOD) diagnosis was confirmed from these findings. Ultrasonographic monitoring reflected the disease status well and demonstrated a complete recovery from the VOD. We emphasize, thus, that abdominal ultrasonography can be applied easily, being a non-invasive procedure, and is useful in diagnosing VOD. Furthermore, the procedure can be repeated serially for evaluating the severity of VOD. Although engraftment was confirmed with granulocytes exceeding 500/microliters, platelet recovery was delayed; the megakaryocytes had not decreased in bone marrow aspirates and platelet-associated IgG was significantly elevated. Since autoimmune thrombocytopenia was highly suspected, to prevent immunosuppression danazol was given as an immune modulator instead of prednisolone, and a complete recovery was obtained. Accordingly, danazol can be used as an alternative to prednisolone for the treatment of autoimmune thrombocytopenia after bone marrow transplantation.- - - - - - - - - - ranking = 1keywords = alternative (Clic here for more details about this article) |
3/5. Membranous obstruction of hepatic venous flow.Membranous obstruction of the inferior vena cava at the suprahepatic level is rare. The present report summarizes direct operative repair. A review of alternative therapeutic strategies is presented.- - - - - - - - - - ranking = 1keywords = alternative (Clic here for more details about this article) |
4/5. Successful treatment of hepatic veno-occlusive disease in a peripheral blood progenitor cell transplant patient with a transjugular intrahepatic portosystemic stent-shunt (TIPS).hepatic veno-occlusive disease (VOD) is a common cause of morbidity and mortality after BMT. Although treatment of VOD is primarily supportive, some success has been obtained recently with fibrinolytic therapy. However, for critically ill patients liver transplantation may be the only therapeutic option. Nevertheless, this procedure is associated with high mortality and can only be performed in a minority of cases. The transjugular intrahepatic portosystemic stent-shunt (TIPS) is a non-surgical, side-to-side shunt consisting of an intraparenchymal duct between a main branch of the portal vein and a hepatic vein. In this report we describe a patient who underwent TIPS placement for severe VOD following autologous PBPC transplant. No complications developed and gradual improvement in clinical status and liver function was observed early after this therapy. Nine months after TIPS, the patient is asymptomatic with normal liver function. TIPS provides an interesting alternative to invasive therapies for patients with severe VOD after bone marrow or PBPC transplants.- - - - - - - - - - ranking = 1keywords = alternative (Clic here for more details about this article) |
5/5. Successful treatment of severe azathioprine-induced hepatic veno-occlusive disease in a kidney-transplanted patient with transjugular intrahepatic portosystemic shunt.azathioprine-induced veno-occlusive disease of the liver mainly described after kidney transplantation is as rare as severe with a high mortality due to acute portal hypertension and liver failure. A kidney-transplanted patient with severe azathioprine-induced veno-occlusive disease of the liver and worsening despite drug discontinuation was treated by emergency transjugular intrahepatic portosystemic shunt. Whereas the veno-occlusive disease was controlled, the patient developed severe intractable portosystemic encephalopathy successfully treated by a stent reducer maintaining a certain degree of portal diversion. Twelve months after transjugular intrahepatic portosystemic shunt, liver function was normalized and the stent was thrombosed with a subnormal liver histology. Thirty-six months after transjugular intrahepatic portosystemic shunt the patient is alive with normal liver function tests and kidney graft function. Transjugular intrahepatic portosystemic shunt for treatment of severe veno-occlusive disease of the liver is an alternative to tide the patient over until recovery of liver function.- - - - - - - - - - ranking = 1keywords = alternative (Clic here for more details about this article) |