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					Stem Cell Transplant Programme
Royal Hospital for Sick Children, Glasgow



      Document Title:                  Veno-Occlusive Disease: Diagnosis and
                                       Management
      Document Type:                   Clinical
      Document No/Version:             YBMT-CLIN-0031

Author:                            Signature: Dr B E S Gibson                   Date: 25/02/10
Dr B E S Gibson
Authorised by:                     Signature: Dr B E S Gibson                   Date: 25/02/10
(Programme Director or
designated individual)
Authorised by:                     Signature: Ms A McVeigh                      Date: 25/02/10
(Quality Manager)
Issue Date:                        03/03/10
Date Effective From:               22/03/10
Review:                            Every two years




      Detail of Amendments Made to Previous Version

Paragraph/Line           Amendment                                 Signature               Date




Distribution          No. of Copies      Issued to (signature)          Issued by              Date
Sch Ward              1                                                 A McVeigh              03/03/10
Sch Daycare           1                                                 A McVeigh              03/03/10
Sch Drs Rm            1                                                 A McVeigh              03/03/10
Clinical Guidelines   1 - Electronic                                    A McVeigh              03/03/10
Website




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       THE DOCUMENT CONTROL SHEET TO BE COMPLETED BY QUALITY MANAGER
                         / DESIGNATED INDIVIDUAL




      Veno-Occlusive Disease:          Version: 1                       Page 1 of 4
      Diagnosis & Management
      Author: Dr B E S Gibson          Ratified: Dr B E S Gibson        Issue Date:   February 2010
      Review Date: February 2012       Ref: YBMT-CLIN-0031
Stem Cell Transplant Programme
Royal Hospital for Sick Children, Glasgow




                   Veno-Occlusive Disease (VOD): Diagnosis and Management


     1. INTRODUCTION

        VOD is a clinical syndrome caused by non-thrombotic obstruction of small
        intrahepatic venules and damage to the surrounding centrilobular hepatocytes
        and sinusoids. It generally occurs within the first 3 weeks of transplant and is
        characterised by jaundice, hepatomegaly and fluid retention with weight gain or
        ascites.

        The diagnosis relies on clinical criteria +/- histological findings on liver biopsy.
        However liver biopsy is rarely performed at this time because of the risk of
        haemorrhage associated with low platelets or abnormal coagulation. Doppler
        studies may be helpful and show reduced or reversed flow in the portal vessels.


     2. RELATED DOCUMENTATION

        None.

     3. AUTHORISED PERSONNEL/SPECIFIC STAFF COMPETENCIES

        The Transplant Consultant / Associate Specialist are responsible for diagnosis and
        management of VOD.

     4. EQUIPMENT/MATERIALS

        None.


     5. PROCEDURE

        5.1 Risk Factors:

            The risk factors for the development of VOD are as follows:
            •       Abnormal liver function, in particular a raised AST, prior to SCT.
            •       Previous liver disease.
            •       Previous abdominal irradiation.
            •       Busulphan containing conditioning regime.
            •       Unrelated donor transplant.
            •       Previous Mylotarg.




     Veno-Occlusive Disease:       Version: 1                    Page 2 of 4
     Diagnosis & Management
     Author: Dr B E S Gibson       Ratified: Dr B E S Gibson     Issue Date:   February 2010
     Review Date: February 2012    Ref: YBMT-CLIN-0031
Stem Cell Transplant Programme
Royal Hospital for Sick Children, Glasgow



     5.2    Symptoms of VOD:
            5.2.1 Classical VOD – this is characterised by jaundice, hepatomegaly +/-
                  right upper quadrant pain and weight gain with oedema and ascites.
                  This occurs within the first two-three weeks after conditioning starts
                  (D+1- D+14/D21).
            5.2.2 Late VOD – this has the same clinical presentation as classical VOD but
                  develops later (Day +30 onwards).
            5.2.3 VOD with multi-organ failure – this has the same clinical presentation
                  as classical and late VOD but with the addition of thrombocytopenia,
                  pleural effusions, pulmonary infiltrates and/or progressive organ
                  failure.

     5.3    Diagnostic Criteria:
            The table below details the Seattle and Baltimore Scoring Criteria, which is
            used in the diagnostic criteria.


                             Seattle Scoring                              Baltimore Scoring

                Diagnosis requires 2 of 3 criteria, occurring    Hyperbilirubinemia plus at least 2 of
                within 20 days of transplantation:               3 other findings:
             -      Bilirubin > 34.2 µmol (2mg/dL)               -   Bilirubin > 34.2 µmol (2mg/dL)
             -      Hepatomegaly       and     upper       right -   Hepatomegaly (usually painful)
                    quadrant pain of liver origin
             -      >2% weight       gain     due     to   fluid -   >5% weight gain Ascites
                    accumulation



     5.4    Differential Diagnosis:
            The following should be excluded prior to making a diagnosis of VOD:
            •         Sepsis with renal impairment
            •         Cholestasis due to TPN
            •         Drug toxicity e.g. cyclosporin, azoles, methotrexate.
            •         Haemolysis
            •         Congestive cardiac failure
            •         Acute Graft versus Host Disease

     5.5    Prophylaxis:
            5.5.1 This is considered in patients at high risk of veno-occlusive disease.
            5.5.2 Prophylactic Ursodeoxycholic acid is given to all allogeneic stem cell
                  transplant recipients within the SCT Programme.
     Veno-Occlusive Disease:         Version: 1                      Page 3 of 4
     Diagnosis & Management
     Author: Dr B E S Gibson         Ratified: Dr B E S Gibson       Issue Date:   February 2010
     Review Date: February 2012      Ref: YBMT-CLIN-0031
Stem Cell Transplant Programme
Royal Hospital for Sick Children, Glasgow



     5.6         Treatment of VOD (Moderate and Severe):
                 5.6.1 Strict fluid balance monitoring and a minimum of twice daily weights.
                 5.6.2 Restrict salt and water intake +/- diuretics.


                 5.6.3 Maintain intravascular volume and renal perfusion by means of
                       albumin, plasma expanders and transfusions (haematocrit >30%).
                 5.6.4 Monitor liver function and correct coagulopathy.
                 5.6.5 Defibrotide will be considered at the discretion of the senior medical
                       staff. Normal dose 20-40mg/kg/per day in 2-4 divided doses.


     6. REFERENCES

           6.1    Carreras E, Granena A, Navasa M, et al. On the reliability of clinical criteria
                  for the diagnosis of hepatic veno-occlusive disease. Ann Hematol. Feb
                  1993;66(2):77-80

           6.2    McDonald GB, Hinds MS, Fisher LD, et al. Veno-occlusive disease of the liver
                  and multi-organ failure after bone marrow transplantation: a cohort study of
                  355 patients. Ann Intern Med. Feb 15 1993;118(4):255-67

           6.3    Jones RJ, Lee KS, Beschorner WE, et al. Veno-occlusive disease of the liver
                  following   bone    marrow    transplantation.    Transplantation.     Dec
                  1987;44(6):778-83


     7. AUDIT AND REVIEW PROCESS

           This SOP will be reviewed every two years.


     8. FURTHER INFORMATION/EXCEPTIONS

           Any planned or unplanned deviations from this SOP must be documented and
           reviewed by the appropriate personnel (see SOP for Non-Conformances and
           Corrective Actions ref YBMT-QUAL-0003)

           For further information contact

           Stem Cell Transplant Team
           Ext: 81880 / 81879 / 89304


     9. APPENDICES

           None.

     Veno-Occlusive Disease:           Version: 1                      Page 4 of 4
     Diagnosis & Management
     Author: Dr B E S Gibson           Ratified: Dr B E S Gibson       Issue Date:   February 2010
     Review Date: February 2012        Ref: YBMT-CLIN-0031