
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Sinusoidal obstruction syndrome natural history, complications and prognosis
Introduction
Overview
Overview
Patients with a mild or moderate form of sinusoidal obstruction syndrome require no specific therapy and can be managed with supportive care alone.The mild and moderate form of sinusoidal obstruction syndrome has a good prognosis. If left untreated, the severe form of sinusoidal obstruction syndrome is characterized by high mortality and progression to multiorgan failure.
Natural History
Natural History
- Sinusoidal obstruction syndrome is a common complication in patients with hematopoietic stem cell transplant.
- If left untreated, the severe form of sinusoidal obstruction syndrome is characterized by high mortality and progression to multiorgan failure.[1]
Complications
Complications
Complications that can develop as a result of sinusoidal obstruction syndrome are:[2][3][4][5]
Prognosis
Prognosis
- Sinusoidal obstruction syndrome is considered mild, moderate or severe based on the clinical course during the first three months of hematopoietic cell transplantation.[6]
- Patients with a mild or moderate form of sinusoidal obstruction syndrome require no specific therapy and can be managed with supportive care alone and progressively resolves over a 2- to 3-week period.[7]
- Patients with the severe sinusoidal obstruction syndrome have a high rate of mortality and can progress to multiorgan failure.[8]
Morbidity and mortality is associated with following factors:[9][10][11][12]
- Younger age in children
- Older age in adults
- Poor performance status
- Pre-existing hepatic dysfunction
- History of pancreatitis
- Advanced malignancy
- Acute leukemia
- Presence of acute graft-versus-host disease
- Allogeneic HCT
- Unrelated donor HCT
- Mismatched donor
- Prior abdominal irradiation
- High dose cytoreductive therapy
- Total body irradiation
References
References
- β Coppell JA, Richardson PG, Soiffer R, Martin PL, Kernan NA, Chen A; et al. (2010). “Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome”. Biol Blood Marrow Transplant. 16 (2): 157β68. doi:10.1016/j.bbmt.2009.08.024. PMCΒ 3018714. PMIDΒ 19766729.
- β Grus T, Lambert L, GrusovΓ‘ G, Banerjee R, BurgetovΓ‘ A (2017). “Budd-Chiari Syndrome”. Prague Med Rep. 118 (2β3): 69β80. doi:10.14712/23362936.2017.6. PMIDΒ 28922103.
- β Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y (2017). “Liver cirrhosis caused by chronic Budd-Chiari syndrome”. Medicine (Baltimore). 96 (34): e7425. doi:10.1097/MD.0000000000007425. PMCΒ 5571988. PMIDΒ 28834866.
- β Lane ER, Hsu EK, Murray KF (2015). “Management of ascites in children”. Expert Rev Gastroenterol Hepatol. 9 (10): 1281β92. doi:10.1586/17474124.2015.1083419. PMIDΒ 26325252.
- β Johnson DB, Savani BN (2012). “How can we reduce hepatic veno-occlusive disease-related deaths after allogeneic stem cell transplantation?”. Exp Hematol. 40 (7): 513β7. doi:10.1016/j.exphem.2012.04.004. PMIDΒ 22542577.
- β McDonald GB, Hinds MS, Fisher LD, Schoch HG, Wolford JL, Banaji M, Hardin BJ, Shulman HM, Clift RA (1993). “Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients”. Ann. Intern. Med. 118 (4): 255β67. PMIDΒ 8420443.
- β Fan CQ, Crawford JM (2014). “Sinusoidal obstruction syndrome (hepatic veno-occlusive disease)”. J Clin Exp Hepatol. 4 (4): 332β46. doi:10.1016/j.jceh.2014.10.002. PMCΒ 4298625. PMIDΒ 25755580.
- β Lopes JA, Jorge S (2011). “Acute kidney injury following HCT: incidence, risk factors and outcome”. Bone Marrow Transplant. 46 (11): 1399β408. doi:10.1038/bmt.2011.46. PMIDΒ 21383682.
- β Tabbara IA, Ghazal CD, Ghazal HH (1996). “Early drop in protein C and antithrombin III is a predictor for the development of venoocclusive disease in patients undergoing hematopoietic stem cell transplantation”. J Hematother. 5 (1): 79β84. doi:10.1089/scd.1.1996.5.79. PMIDΒ 8646485.
- β Salat C, Holler E, Kolb HJ, Reinhardt B, Pihusch R, Wilmanns W, Hiller E (1997). “Plasminogen activator inhibitor-1 confirms the diagnosis of hepatic veno-occlusive disease in patients with hyperbilirubinemia after bone marrow transplantation”. Blood. 89 (6): 2184β8. PMIDΒ 9058743.
- β Faioni EM, Krachmalnicoff A, Bearman SI, Federici AB, Decarli A, Gianni AM, McDonald GB, Mannucci PM (1993). “Naturally occurring anticoagulants and bone marrow transplantation: plasma protein C predicts the development of venocclusive disease of the liver”. Blood. 81 (12): 3458β62. PMIDΒ 8507881.
- β Scrobohaci ML, Drouet L, Monem-Mansi A, Devergie A, Baudin B, D’Agay MF, Gluckman E (1991). “Liver veno-occlusive disease after bone marrow transplantation changes in coagulation parameters and endothelial markers”. Thromb. Res. 63 (5): 509β19. PMIDΒ 1755004.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Husnain Shaukat, M.D [2]
