VOD, also known as sinusoidal obstruction syndrome, begins in the liver and can quickly affect other vital organs, most notably the kidneys and lungs.1,2,3 It is a rare and potentially deadly complication of hematopoietic stem cell transplantation (HSCT) occurring in approximately 9-14% of HSCT patients.1,4
HSCTs, during which a patient receives either their own or donor stem cells after high-dose chemotherapy, are performed to treat blood cancer.5 The procedure “rescues” patients from therapy-induced immune system suppression and contributes to ongoing cancer eradication.5 Treatments given to prepare bone marrow for a stem-cell transplant are called conditioning regimens and may include chemotherapy or radiation.2,6,7 These treatments can damage cells in the liver, leading to a blockage that can reduce blood flow in the liver. This can cause changes throughout the body.2,6,7
While patients undergo stem cell transplants with the intent of improving their health outcomes – including those with hematological malignancies and some non-malignant disorders8,9 – VOD can develop without warning and progress rapidly, causing severe kidney or lung dysfunction that can lead to organ failure.1,2,3,10 It is thought that the initial trigger for the development of VOD is damage to cells in the liver, called hepatocytes, and activation of sinusoidal endothelial cells, also located in the liver.7,11,12 This is caused by toxic metabolites, or small molecules, generated during the conditioning regimen used prior to transplantation.7,13,14
VOD can be treatable.2 Understanding the signs and symptoms and how to recognize them can help patients and their health care professionals determine the appropriate path forward as soon as possible.2 The following information outlines pre-existing factors that may increase a person’s risk for VOD, along with signals of potentially life-threatening VOD progression.
VOD can develop unexpectedly in already vulnerable cancer patients who have undergone HSCT, so vigilant monitoring by the transplant team for signs of organ dysfunction for the first 21 days post-transplant is critical to its detection.2 These signs can include7:
While VOD can occur in any patient following HSCT, specific groups of HSCT patients, including children, people who have suffered a previous injury to the liver and recipients of allogenic (i.e., from another individual) hematopoietic stem cells, are at higher risk of developing VOD.4
In approximately 30-50% of patients diagnosed with VOD, the disease can progress rapidly and lead to the development multi-organ dysfunction, and when it does, it can be fatal.15, a And although VOD appears most often within three weeks post-HSCT, onset can also occur later in the course of recovery, even after discharge from the hospital.7 That’s why being aware of the symptoms and monitoring for signs of life-threatening progression of VOD are so important.3,4,15
U.S. HCPs can learn more about the unpredictability, signs of progression and potentially life-threatening consequences of VOD by visiting www.ProgressiveVOD.com.
aBased on a study conducted by Carreras et al that used 2 sets of diagnostic criteria to estimate the incidence of VOD after HSCT.
1Tsirigotis PD, Resnick IB, Avni B, et al. Incidence and risk factors for moderate-to-severe veno-occlusive disease of the liver after allogeneic stem cell transplantation using a reduced intensity conditioning regimen. Bone Marrow Transplant. 2014;49(11):1389-1392.
2Richardson, PG, Cooke KR, Krishnan AY, et al. Strategies for the Management of Hepatic Veno-Occlusive Disease in Patients Undergoing Bone Marrow Transplant. Clinical Advances in Hematology & Oncology. 2015;13(12):12.
3McDonald GB, Hinds MS, Fisher LD, et al. Venoocclusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients. Ann Intern Med. 1993;118(4):255-267.
4Coppell JA, Richardson PG, Soiffer R, et al. Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome. Biol Blood Marrow Transplant. 2010;16(2):157-168.
5Hatzimichael E, Tuthill M. Hematopoietic stem cell transplantation. Stem Cells Cloning. 2010;3:105–117. Published 2010 Aug 26. doi:10.2147/SCCAA.S6815
6Ikehara S. New strategies for BMT and organ transplantation. Int J Hematol. 2002;76(Suppl 1):161-4.
7Carreras E. Early complications after HSCT. In: Apperley J, Carreras E, Gluckman E, et al, eds. The EBMT Handbook. 6th ed. Paris, France: European School of Haematology; 2012:176-195.
8Mohty M, Malard F, Abecassis M, et al. Sinusoidal obstruction syndrome/veno-occlusive disease: current situation and perspectives—a position statement from the European Society for Blood and Marrow Transplantation (EBMT). Bone Marrow Transplant. 2015;50(6):781-789.
9Mohty M, Malard F, Abecassis M, et al. 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. 2016;51(7):906-912.
10Bearman SI. The syndrome of hepatic veno-occlusive disease after marrow transplantation. Blood. 1995;85(11):3005-3020.
11Richardson PG, Ho VT, Cutler C, et al. Hepatic veno-occlusive disease after hematopoietic stem cell transplantation: novel insights to pathogenesis, current status of treatment, and future directions. Biol Blood Marrow Transplant. 2013;19(suppl 1):S88-S90.
12Richardson PG, Corbacioglu S, Ho VT, et al. Exper Opin Drug Saf. 2013;12(1):123-136.
13Richardson PG, Ho VT, Cutler C, et al. Hepatic veno-occlusive disease after hematopoietic stem cell transplantation: novel insights to pathogenesis, current status of treatment, and future directions. Biol Blood Marrow Transplant. 2013;19(suppl 1):S88-S90.
14Richardson PG, Corbacioglu S, Ho VT, et al. Exper Opin Drug Saf. 2013;12(1):123-136.
15Carreras E, Díaz-Beyá M, Rosiñol L, et al. The incidence of veno-occlusive disease following allogeneic hematopoietic stem cell transplantation has diminished and the outcome improved over the last decade. Biol Blood Marrow Transplant. 2011;17(11):1713-1720.
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