Sinusoidales Obstruktionssyndrom und venookklusive Lebererkrankung – Diagnose und Therapiemöglichkeiten

Hepatic sinusoidal obstruction syndrome—diagnostics and therapy

Zusammenfassung

Das sinusoidale Obstruktionssyndrom (SOS), auch als „veno-occlusive disease“ (VOD) bezeichnet, ist eine seltene, potenziell letal verlaufende Durchblutungsstörung der Lebersinusoide, der ursächlich in den meisten Fällen eine toxische Schädigung im Rahmen einer Stammzelltransplantation vorangeht. Die Diagnose ist oft schwierig und basiert auf klinischen Kriterien sowie auf Laborparametern und Bildgebung. Klinische Parameter sind unter anderem Ikterus, Gewichtszunahme und eine schmerzhafte Hepatomegalie. Bildgebend ist v. a. die abdominelle Sonographie von Bedeutung. Therapeutisch werden milde Verläufe supportiv behandelt, bei schwer erkrankten Patienten hat ein frühzeitiger Therapiebeginn mit Defibrotid positive Effekte gezeigt.

Abstract

Hepatic sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease (VOD), is a rare, potentially life-threatening microcirculatory disorder of the hepatic sinuses that in most cases occurs after a prior hematopoietic stem cell transplantation. Diagnosis is often difficult and based on clinical criteria, laboratory parameters and imaging. Clinical parameters are, among others, jaundice, weight gain and painful hepatomegaly. Abdominal ultrasound serves as an important imaging tool. Mild courses receive symptomatic treatment, while for severe cases the early administration of defibrotide has shown positive effects.

This is a preview of subscription content, access via your institution.

Abb. 1
Abb. 2
Abb. 3
Abb. 4

Literatur

  1. 1.

    Anonymous (2016) EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol 64:179–202

    Article  Google Scholar 

  2. 2.

    Colecchia A, Ravaioli F, Sessa M et al (2019) Liver stiffness measurement allows early diagnosis of Veno-occlusive disease/sinusoidal obstruction syndrome in adult patients who undergo hematopoietic stem cell transplantation: results from a monocentric prospective study. Biol Blood Marrow Transplant 25:995–1003

    Article  Google Scholar 

  3. 3.

    Coppell JA, Richardson PG, Soiffer R et al (2010) Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome. Biol Blood Marrow Transplant 16:157–168

    Article  Google Scholar 

  4. 4.

    Corbacioglu S, Cesaro S, Faraci M et al (2012) Defibrotide for prophylaxis of hepatic veno-occlusive disease in paediatric haemopoietic stem-cell transplantation: an open-label, phase 3, randomised controlled trial. Lancet 379:1301–1309

    CAS  Article  Google Scholar 

  5. 5.

    Corbacioglu S, Greil J, Peters C et al (2004) Defibrotide in the treatment of children with veno-occlusive disease (VOD): a retrospective multicentre study demonstrates therapeutic efficacy upon early intervention. Bone Marrow Transplant 33:189–195

    CAS  Article  Google Scholar 

  6. 6.

    Corbacioglu S, Jabbour EJ, Mohty M (2019) Risk factors for development of and progression of hepatic veno-occlusive disease/sinusoidal obstruction syndrome. Biol Blood Marrow Transplant 25:1271–1280

    Article  Google Scholar 

  7. 7.

    Deleve LD, Shulman HM, Mcdonald GB (2002) Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease). Semin Liver Dis 22:27–42

    Article  Google Scholar 

  8. 8.

    Dietrich CF, Trenker C, Fontanilla T et al (2018) New ultrasound techniques challenge the diagnosis of sinusoidal obstruction syndrome. Ultrasound Med Biol 44:2171–2182

    Article  Google Scholar 

  9. 9.

    Erturk SM, Mortelé KJ, Binkert CA et al (2006) CT features of hepatic venoocclusive disease and hepatic graft-versus-host disease in patients after hematopoietic stem cell transplantation. AJR Am J Roentgenol 186:1497–1501

    Article  Google Scholar 

  10. 10.

    Fan CQ, Crawford JM (2014) Sinusoidal obstruction syndrome (hepatic veno-occlusive disease). J Clin Exp Hepatol 4:332–346

    Article  Google Scholar 

  11. 11.

    Fontanilla T, Hernando CG, Claros JC et al (2011) Acoustic radiation force impulse elastography and contrast-enhanced sonography of sinusoidal obstructive syndrome (Veno-occlusive Disease): preliminary results. J Ultrasound Med 30:1593–1598

    Article  Google Scholar 

  12. 12.

    Imran H, Tleyjeh IM, Zirakzadeh A et al (2006) Use of prophylactic anticoagulation and the risk of hepatic veno-occlusive disease in patients undergoing hematopoietic stem cell transplantation: a systematic review and meta-analysis. Bone Marrow Transplant 37:677–686

    CAS  Article  Google Scholar 

  13. 13.

    Jones RJ, Lee KS, Beschorner WE et al (1987) Venoocclusive disease of the liver following bone marrow transplantation. Transplantation 44:778–783

    CAS  Article  Google Scholar 

  14. 14.

    Kernan NA, Grupp S, Smith AR et al (2018) Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome. Br J Haematol 181:816–827

    CAS  Article  Google Scholar 

  15. 15.

    Mahgerefteh SY, Sosna J, Bogot N et al (2011) Radiologic imaging and intervention for gastrointestinal and hepatic complications of hematopoietic stem cell transplantation. Radiology 258:660–671

    Article  Google Scholar 

  16. 16.

    Mcdonald GB, Sharma P, Matthews DE et al (1984) Venocclusive disease of the liver after bone marrow transplantation: diagnosis, incidence, and predisposing factors. Hepatology 4:116–122

    CAS  Article  Google Scholar 

  17. 17.

    Mohty M, Malard F, Abecassis M et al (2016) Revised diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a new classification from the European Society for Blood and Marrow Transplantation. Bone Marrow Transplant 51:906–912

    CAS  Article  Google Scholar 

  18. 18.

    Nassereddine S, Alsubait S, Tabbara I (2018) Sinusoidal obstruction syndrome (Veno-occlusive disease) following hematopoietic stem cell transplant: insights and therapeutic advances. Anticancer Res 38:2597–2605

    CAS  PubMed  Google Scholar 

  19. 19.

    Ohashi K, Tanabe J, Watanabe R et al (2000) The Japanese multicenter open randomized trial of ursodeoxycholic acid prophylaxis for hepatic veno-occlusive disease after stem cell transplantation. Am J Hematol 64:32–38

    CAS  Article  Google Scholar 

  20. 20.

    Richardson PG, Riches ML, Kernan NA et al (2016) Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure. Blood 127:1656–1665

    CAS  Article  Google Scholar 

  21. 21.

    Richardson PG, Soiffer RJ, Antin JH et al (2010) Defibrotide for the treatment of severe hepatic veno-occlusive disease and multiorgan failure after stem cell transplantation: a multicenter, randomized, dose-finding trial. Biol Blood Marrow Transplant 16:1005–1017

    CAS  Article  Google Scholar 

  22. 22.

    Ruutu T, Eriksson B, Remes K et al (2002) Ursodeoxycholic acid for the prevention of hepatic complications in allogeneic stem cell transplantation. Blood 100:1977–1983

    CAS  Article  Google Scholar 

  23. 23.

    Schulz M, Tacke F (2020) Identifying high-risk NASH patients: what we know so far. Hepat Med 12:125–138

    Article  Google Scholar 

  24. 24.

    Shin NY, Kim MJ, Lim JS et al (2012) Accuracy of gadoxetic acid-enhanced magnetic resonance imaging for the diagnosis of sinusoidal obstruction syndrome in patients with chemotherapy-treated colorectal liver metastases. Eur Radiol 22:864–871

    Article  Google Scholar 

  25. 25.

    Shulman HM, Fisher LB, Schoch HG et al (1994) Veno-occlusive disease of the liver after marrow transplantation: histological correlates of clinical signs and symptoms. Hepatology 19:1171–1181

    CAS  Article  Google Scholar 

  26. 26.

    Soubrane O, Brouquet A, Zalinski S et al (2010) Predicting high grade lesions of sinusoidal obstruction syndrome related to oxaliplatin-based chemotherapy for colorectal liver metastases: correlation with post-hepatectomy outcome. Ann Surg 251:454–460

    Article  Google Scholar 

  27. 27.

    Trenker C, Burchert A, Schumacher C et al (2020) Pathologic hepatic contrast-enhanced ultrasound pattern in patients undergoing allogeneic stem cell transplantation. Ultrasound Med Biol 46:1865–1871

    Article  Google Scholar 

  28. 28.

    Valla DC, Cazals-Hatem D (2016) Sinusoidal obstruction syndrome. Clin Res Hepatol Gastroenterol 40:378–385

    Article  Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to M. Schulz.

Ethics declarations

Interessenkonflikt

M. Schulz und A. Wree erklären, dass sie sich bei der Erstellung des Beitrags nicht von wirtschaftlichen Interessen leiten ließen. Es werden die folgenden potenziellen Interessenkonflikte offengelegt: Forschungsarbeiten im Labor von Prof. Wree wurde von Intercept gefördert, er hat Vortrags- oder Beratungshonorare von Falk, Intercept, Gilead und Daiichi-Sankyo erhalten.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

Additional information

Redaktion

J. Mayerle, München

F. Tacke, Berlin

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Schulz, M., Wree, A. Sinusoidales Obstruktionssyndrom und venookklusive Lebererkrankung – Diagnose und Therapiemöglichkeiten. Gastroenterologe (2021). https://doi.org/10.1007/s11377-021-00505-7

Download citation

Schlüsselwörter

  • Lebererkrankungen
  • Gefäßerkrankungen
  • Ikterus
  • Sonographie
  • Elastographie

Keywords

  • Liver diseases
  • Vascular diseases
  • Jaundice
  • Ultrasonography
  • Elastography