Sinusoidal obstruction syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Patients with a mild or moderate form of sinusoidal obstruction syndrome require no specific therapy and can be managed with supportive care alone and generally 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
- 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 that can develop as a result of sinusoidal obstruction syndrome are:[2][3][4][5]
- Portal hypertension
- Hepatic encephalopathy
- Variceal hemorrhage
- Hepatorenal syndrome
- Hepatic decompensation
- Bacterial peritonitis especially the following paracentesis
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 requires 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 has 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
- ↑ 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): 157–68. doi:10.1016/j.bbmt.2009.08.024. PMC 3018714. PMID 19766729.
- ↑ Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A (2017). "Budd-Chiari Syndrome". Prague Med Rep. 118 (2–3): 69–80. doi:10.14712/23362936.2017.6. PMID 28922103.
- ↑ 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. PMC 5571988. PMID 28834866.
- ↑ Lane ER, Hsu EK, Murray KF (2015). "Management of ascites in children". Expert Rev Gastroenterol Hepatol. 9 (10): 1281–92. doi:10.1586/17474124.2015.1083419. PMID 26325252.
- ↑ Johnson DB, Savani BN (2012). "How can we reduce hepatic veno-occlusive disease-related deaths after allogeneic stem cell transplantation?". Exp Hematol. 40 (7): 513–7. doi:10.1016/j.exphem.2012.04.004. PMID 22542577.
- ↑ 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): 255–67. PMID 8420443.
- ↑ Fan CQ, Crawford JM (2014). "Sinusoidal obstruction syndrome (hepatic veno-occlusive disease)". J Clin Exp Hepatol. 4 (4): 332–46. doi:10.1016/j.jceh.2014.10.002. PMC 4298625. PMID 25755580.
- ↑ Lopes JA, Jorge S (2011). "Acute kidney injury following HCT: incidence, risk factors and outcome". Bone Marrow Transplant. 46 (11): 1399–408. doi:10.1038/bmt.2011.46. PMID 21383682.
- ↑ 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): 79–84. doi:10.1089/scd.1.1996.5.79. PMID 8646485.
- ↑ 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): 2184–8. PMID 9058743.
- ↑ 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): 3458–62. PMID 8507881.
- ↑ 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): 509–19. PMID 1755004.