Budd-chiari syndrome in patients with hematological disease: A therapeutic challenge

Ganguli, Sujata; Ramzan, Nizar N.; McKusick, Michael A.; Andrews, James C.; Phyliky, Robert L.; Kamath, Patrick S.

doi:10.1002/hep.510270434

Cited by 41 publications

(12 citation statements)

References 26 publications

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“…Although our study failed to show the impact of the underlying disease on outcome, a longer follow-up may have revealed a difference between patients with or without a myeloproliferative syndrome. Myeloproliferative disorders such as polycythaemia rubra vera are slow diseases with a rate of leukaemic transformation of 10% at 15 years and 25% at 25 years, and a nearly normal 10-year life expectancy [15]. Our study, however, shows that the slow course of myeloproliferative syndromes is not appreciably affected by transplantation, and that LT can be offered to these patients in spite of the possible negative effect in the long term.…”

Section: Discussionmentioning

confidence: 66%

Liver transplantation for Budd–Chiari syndrome: A European study on 248 patients from 51 centres

Mentha

Giostra

Majno

et al. 2006

Journal of Hepatology

208

134

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Section: Discussionmentioning

confidence: 66%

Liver transplantation for Budd–Chiari syndrome: A European study on 248 patients from 51 centres

Mentha

Giostra

Majno

et al. 2006

Journal of Hepatology

208

134

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“…This approach should be attempted in early diagnosed cases, especially in those detected in the initial 24 to 72 hours, 2,26,27 although it is worth trying in patients with hepatic vein thrombosis less than 2 weeks old. 28 Medical treatment, including therapy of the frequent underlying hematologic disease, anticoagulation, and diuretics have for a long time been considered completely ineffective and associated with very poor long-term results. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Indeed, a 2-year survival lower than 10% was reported in medically treated patients, 29,30 and recent surgical reports consider portal decompressive therapy as the only possible approach except from liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy

et al. 2002

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Patients with Budd-Chiari syndrome (BCS) may require treatment with portal decompressive surgery or liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) represents a new treatment alternative, but its long-term effect on BCS outcome has not been evaluated. Twenty-one patients with BCS consecutively admitted to our unit were evaluated. The mean follow-up was 4 ؎ 3 years. Seven patients had nonprogressive forms and were successfully controlled with medical therapy; 1 case, with a short-length hepatic vein stenosis was successfully treated by angioplasty. All 8 patients are alive and asymptomatic. The remaining 13 patients, had a TIPS because of clinical deterioration (in one of them, because early TIPS thrombosis a successful side-to-side portacaval shunt [SSPCS] was performed) followed by an improvement in clinical condition. However, a patient with fulminant liver failure before TIPS insertion, died 4 months later and another patient with cirrhosis at diagnosis had liver transplantation 2 years later. The remaining 11 patients are alive and free of ascites. In 3 of these patients TIPS is patent after 3, 6, and 12 months. The remaining 8 patients developed late TIPS dysfunction. In two of these cases, after angioplasty and restenting, TIPS is patent after a follow-up of 9 and 80 months. In 5 other patients, recurring TIPS occlusion was not further corrected because no signs of portal hypertension were present. In conclusion, in patients with BCS uncontrolled with medical therapy, TIPS is a highly effective technique that is associated with long-term survival. (HEPATOLOGY 2002;35: 132-139.)

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“…In a more recent presentation, diuretics, paracentesis, and a sodium-restricted diet were considered useful supportive therapy in any stage of the disease. Thrombolytic therapy is suggested in the case of an onset of the disease that lies no longer than 2-3 weeks behind (9). Tilanus summarizes that anticoagulants should be used to avoid progression of the thrombotic disease; resolution of the thombotic clots by anticoagulants was not assumed (1).…”

Section: Tailored Management According To the Stage Of The Diseasementioning

confidence: 99%

“…12). A trial with anticoagulant therapy is recommended in patients with an onset less than 2 weeks previously (9).…”

Section: Perioperative Managementmentioning

confidence: 99%

Management of Budd?Chiari Syndrome

et al. 2005

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Thrombotic occlusion of the hepatic veins leads to liver dysfunction and liver failure requiring liver transplantation in advanced cases. The cause for the occlusion of the hepatic veins is not completely understood. However, several underlying conditions such as polycytemia, factor V Leiden mutation, and protein C and S deficiency are found in these patients. We here report our single-center experience with 18 consecutive patients with Budd-Chiari Syndrome (BCS) who were treated at our institution between August 1992 and June 2003. Twelve patients underwent liver transplantation, three patients received stents into the hepatic veins or vena cava, another patient was treated with TIPSS (transjugular intrahepatic postosystemic stent shunt), and one patient underwent surgical mesocaval shunting. Three patients, among those the patient with TIPSS, were put on anticoagulant therapy and are scheduled for liver transplantation. We outline the indication for an approach tailored to the stage of the disease and the adaption of the procedures with the deterioration of clinical conditions. Surgical aspects and postoperative management with a focus on liver transplantation are outlined. We conclude from our observations that the management of BCS requires an approach that exhausts conservative approaches until clinical conditions deteriorate with respect to portal hypertension or liver function. Conservative management, i.e., interventional and supportive medical therapy, has been used up to 8 years in our series, until the time for liver transplantation is reached. Liver transplantation for BCS had more complications than transplantation for other liver diseases in our series. Therefore, we propose to keep liver function stable using interventional techniques to maintain venous outflow. If venous outflow cannot be interventionally restored and liver function deteriorates or cirrhosis develops during this time course, liver transplantation is the therapy of choice.

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Budd-chiari syndrome in patients with hematological disease: A therapeutic challenge

Cited by 41 publications

References 26 publications

Liver transplantation for Budd–Chiari syndrome: A European study on 248 patients from 51 centres

Liver transplantation for Budd–Chiari syndrome: A European study on 248 patients from 51 centres

TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy

Management of Budd?Chiari Syndrome

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