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BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation.
Br J Haematol. 2013 Nov; 163(4):444-57.BJ

Abstract

DIAGNOSIS

It is recommended that the diagnosis of veno-occlusive disease (sinusoidal obstruction syndrome) [VOD (SOS)] be based primarily on established clinical criteria (modified Seattle or Baltimore criteria) (1A). Ultrasound imaging may be helpful in the exclusion of other disorders in patients with suspected VOD (SOS) (1C). It is recommended that liver biopsy be reserved for patients in whom the diagnosis of VOD (SOS) is unclear and there is a need to exclude other diagnoses (1C). It is recommended that liver biopsies are undertaken using the transjugular approach in order to reduce the risks associated with the procedure (1C). It is suggested that the role of plasminogen activator inhibitor 1 levels remains an area for further research but that these levels should not form part of the routine diagnostic work-up for VOD (SOS) at present (2C).

RISK FACTORS

It is recommended that patients are assessed for risk factors for VOD (SOS) and that these risk factors are addressed prior to haematopoietic stem cell transplantation (1A).

PROPHYLAXIS

Defibrotide is recommended at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in children undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (1A). Defibrotide is suggested at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in adults undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (2B). Prostaglandin E1 is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy and toxicity (1B). Pentoxifylline is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy (1A). Ursodeoxycholic acid is suggested for use in the prophylaxis of VOD (SOS) (2C). Heparin (unfractionated and low molecular weight) is not suggested for use in the prophylaxis of VOD (SOS) due to the risk of increased toxicity (2B). Antithrombin is not suggested for the prophylaxis of VOD (SOS) due to lack of efficacy (2B).

TREATMENT

Defibrotide is recommended in the treatment of VOD (SOS) in adults and children (1B). Tissue plasminogen activator is not recommended for use in the treatment of VOD (SOS) due to the associated risk of haemorrhage (1B). N-acetylcysteine is not routinely recommended for use in the treatment of veno-occlusive disease due to lack of efficacy (1A). Methylprednisolone may be considered for use in the treatment of veno-occlusive disease with the appropriate caveats of caution regarding infection (2C). Judicious clinical care, particularly in the management of fluid balance, is recommended in the management of VOD (SOS) (1C). Early discussion with critical care specialists and a specialist hepatology unit is recommended in the management of VOD (SOS) and other treatment options including transjugular intrahepatic portosystemic shunt or hepatic transplantation may be considered (1C).

SUMMARY

A joint working group established by the Haemato-oncology subgroup of the British Committee for Standards in Haematology (BCSH) and the British Society for Blood and Marrow Transplantation (BSBMT) has reviewed the available literature and made recommendations for the diagnosis and management of veno-occlusive disease of the liver following haematopoietic stem cell transplantation (HSCT). This guideline includes recommendations for both prophylaxis and treatment of the condition and includes recommendations for children and adults undergoing HSCT.

Authors+Show Affiliations

Department of Haematology, Central Manchester NHS Foundation Trust, Manchester, UK.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Practice Guideline

Language

eng

PubMed ID

24102514

Citation

Dignan, Fiona L., et al. "BCSH/BSBMT Guideline: Diagnosis and Management of Veno-occlusive Disease (sinusoidal Obstruction Syndrome) Following Haematopoietic Stem Cell Transplantation." British Journal of Haematology, vol. 163, no. 4, 2013, pp. 444-57.
Dignan FL, Wynn RF, Hadzic N, et al. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. Br J Haematol. 2013;163(4):444-57.
Dignan, F. L., Wynn, R. F., Hadzic, N., Karani, J., Quaglia, A., Pagliuca, A., Veys, P., & Potter, M. N. (2013). BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. British Journal of Haematology, 163(4), 444-57. https://doi.org/10.1111/bjh.12558
Dignan FL, et al. BCSH/BSBMT Guideline: Diagnosis and Management of Veno-occlusive Disease (sinusoidal Obstruction Syndrome) Following Haematopoietic Stem Cell Transplantation. Br J Haematol. 2013;163(4):444-57. PubMed PMID: 24102514.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. AU - Dignan,Fiona L, AU - Wynn,Robert F, AU - Hadzic,Nedim, AU - Karani,John, AU - Quaglia,Alberto, AU - Pagliuca,Antonio, AU - Veys,Paul, AU - Potter,Michael N, AU - ,, AU - ,, Y1 - 2013/09/17/ PY - 2013/10/10/entrez PY - 2013/10/10/pubmed PY - 2014/5/7/medline KW - defibrotide KW - prophylaxis KW - sinusoidal obstruction syndrome KW - transplant KW - veno-occlusive disease SP - 444 EP - 57 JF - British journal of haematology JO - Br. J. Haematol. VL - 163 IS - 4 N2 - DIAGNOSIS: It is recommended that the diagnosis of veno-occlusive disease (sinusoidal obstruction syndrome) [VOD (SOS)] be based primarily on established clinical criteria (modified Seattle or Baltimore criteria) (1A). Ultrasound imaging may be helpful in the exclusion of other disorders in patients with suspected VOD (SOS) (1C). It is recommended that liver biopsy be reserved for patients in whom the diagnosis of VOD (SOS) is unclear and there is a need to exclude other diagnoses (1C). It is recommended that liver biopsies are undertaken using the transjugular approach in order to reduce the risks associated with the procedure (1C). It is suggested that the role of plasminogen activator inhibitor 1 levels remains an area for further research but that these levels should not form part of the routine diagnostic work-up for VOD (SOS) at present (2C). RISK FACTORS: It is recommended that patients are assessed for risk factors for VOD (SOS) and that these risk factors are addressed prior to haematopoietic stem cell transplantation (1A). PROPHYLAXIS: Defibrotide is recommended at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in children undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (1A). Defibrotide is suggested at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in adults undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (2B). Prostaglandin E1 is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy and toxicity (1B). Pentoxifylline is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy (1A). Ursodeoxycholic acid is suggested for use in the prophylaxis of VOD (SOS) (2C). Heparin (unfractionated and low molecular weight) is not suggested for use in the prophylaxis of VOD (SOS) due to the risk of increased toxicity (2B). Antithrombin is not suggested for the prophylaxis of VOD (SOS) due to lack of efficacy (2B). TREATMENT: Defibrotide is recommended in the treatment of VOD (SOS) in adults and children (1B). Tissue plasminogen activator is not recommended for use in the treatment of VOD (SOS) due to the associated risk of haemorrhage (1B). N-acetylcysteine is not routinely recommended for use in the treatment of veno-occlusive disease due to lack of efficacy (1A). Methylprednisolone may be considered for use in the treatment of veno-occlusive disease with the appropriate caveats of caution regarding infection (2C). Judicious clinical care, particularly in the management of fluid balance, is recommended in the management of VOD (SOS) (1C). Early discussion with critical care specialists and a specialist hepatology unit is recommended in the management of VOD (SOS) and other treatment options including transjugular intrahepatic portosystemic shunt or hepatic transplantation may be considered (1C). SUMMARY: A joint working group established by the Haemato-oncology subgroup of the British Committee for Standards in Haematology (BCSH) and the British Society for Blood and Marrow Transplantation (BSBMT) has reviewed the available literature and made recommendations for the diagnosis and management of veno-occlusive disease of the liver following haematopoietic stem cell transplantation (HSCT). This guideline includes recommendations for both prophylaxis and treatment of the condition and includes recommendations for children and adults undergoing HSCT. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/24102514/BCSH/BSBMT_guideline:_diagnosis_and_management_of_veno_occlusive_disease__sinusoidal_obstruction_syndrome__following_haematopoietic_stem_cell_transplantation_ L2 - https://doi.org/10.1111/bjh.12558 DB - PRIME DP - Unbound Medicine ER -