An international survey to better understand the current incidence, severity, and management of VOD/SOS

An international survey to better understand the current incidence, severity, and management of veno-occlusive disease/sinusoidal obstruction syndrome of the liver in adult patients undergoing hematopoietic stem cell transplantation (HSCT)

Veno-occlusive disease or sinusoidal obstruction syndrome of the liver (VOD/SOS) is a major, potentially fatal complication of hematopoietic stem cell transplantation (HSCT), particularly allogeneic transplantation. The course of this complication is variable, but in a large proportion it leads to multiorgan failure with a high mortality rate.

Significant developments in the diagnostics and management of this complication have taken place in recent years. Effective treatment has become available, and some refined diagnostic and severity criteria for VOD/SOS, were recently published.

With this background, the IACH designed this survey to evaluate, the current experience among health care professionals, regarding the incidence of VOD/SOS, the diagnostic procedures, prophylaxis and treatment used, role of the severity grading, and the outcome.

The survey will take less than 10 minutes to complete. We really appreciate your support and value your input as we are continually looking to improve patient care.

20 survey responders will win free registration to the upcoming IACH Annual Meeting which will take place in Paris this coming October 2024.

Thank you, and best wishes

Prof. Mohamad Mohty and Prof Enric Carreras
On behalf of the IACH Scientiﬁc Steering Committee

Question Title

1. What is your profession? (Required.)

Clinical Hematologist

Adult stem cell transplant physician

Pediatric stem cell transplant physician

Intern/resident/fellow/registrar/student

Nurse

Industry representative (MD, PhD, Pharmacist, etc.)

Other (please specify)

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2. How old are you? (Required.)

Less than 30

31-40

41-50

51-60

61 or older

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3. In what country do you practice? (Required.)

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4. How long have you been practicing? (Required.)

Less than a year

1-2 year

3-5 years

6-10 years

Over 10 years

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5. Are you a member of, or aﬀiliated with any of the following national/regional societies/organizations? (Please select all that apply) (Required.)

ASH

EHA

ASCO

EBMT

National transplant society

Other (please specify)

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6. What is your primary focus in transplantation? (Please select all that apply) (Required.)

Conditioning regimens

GVHD prophylaxis

Infectious complications in general

Viral infections

Bacterial infections

Fungal infections

Early transplant complications (eg. VOD; TMA, etc.)

Acute GVHD

Chronic GVHD

Post-transplant disease therapies

Long-term side effects

Quality of life and rehabilitation

Other (please specify)

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7. On average, what percentage of your working time do you spend on the following activities (numbers must add up to 100%)? (Required.)

Patient clinical care

Clinical and/or Translational Research

Academic activities (teaching / learning)

Advocacy / Interest groups / Medical societies

Other

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8. Do you find it difficult to establish/make an early diagnosis of VOD/SOS in your practice? (Required.)

Yes

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9. Please explain your answer to the question above (Required.)

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10. What is the current incidence of liver VOD/SOS in your practice? (Required.)

Less than 1%

1-5%

6-10%

11-15%

>15%

I don’t know the exact incidence because we can frequently miss the diagnosis

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11. Do you see VOD/SOS outside allogeneic hematopoietic cell transplantation? (please select all that apply) (Required.)

Autologous stem cell transplantation

High-dose chemotherapy alone

When using some specific drugs (eg. gemtuzumab, inotuzumab, etc.)

Other (please specify)

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12. Which criteria do you use to establish the diagnosis of VOD/SOS? (please select all that apply) (Required.)

Baltimore criteria alone

Seattle and modified Seattle criteria alone

2016 EBMT criteria (Bone Marrow Transplant. 2016; 51:906-12. https://doi.org/10.1038/bmt.2016.130)

Refined EBMT criteria (Bone Marrow Transplant. 2023 58:749-754. https://doi.org/10.1038/s41409-023-01992-8)

Other (please specify)

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13. The refined EBMT criteria (Bone Marrow Transplant. 2023 58:749-754. https://doi.org/10.1038/s41409-023-01992-8) introduced the concept of PROBABLE, CLASSICAL and PROVEN VOD/SOS. Do you feel this is useful in your practice to improve the diagnostic process of VOD/SOS? (Required.)

Yes

I don’t know/not sure

Other (please specify)

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14. How useful is the PROBABLE hepatic VOD diagnostic category for your clinical practice? (Required.)

Not useful

Useful

Very useful

I don’t know

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15. The unmodifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

Second HSCT

Advanced disease (beyond second CR or relapse)

Primary immunodeficiency diagnosis

Genetic factors (GSTM1 polymorphism, C282Y allele, MTHFR 677CC/1298CC haplotype)

Older patient age

Increased serum transaminase

Karnofsky score below 90%

Metabolic syndrome

Female receiving norethisterone

Previous use of gemtuzumab ozogamicin or inotuzumab ozogamicin

Hepatotoxic drugs

Iron overload (>1.000 ng/mL)

Serum bilirubin > 1.5 mg/l (>26 μmol/l), transaminase >2.5 ULN

Pre‐existing liver disease: hepatic fibrosis, cirrhosis, active viral hepatitis

Abdominal or hepatic irradiation

Question Title

16. The modifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

High dose (myeloablative) regimens

Oral or high dose busulfan

High dose treosulfan

High dose TBI-based regimen

Unrelated donor

HLA-mismatched donor

GVHD prophylaxis with sirolimus + methotrexate + tacrolimus

GVHD prophylaxis with methotrexate + cyclosporin or tacrolimus

Non T-cell depleted transplant

Use of parenteral nutrition

Question Title

17. The severity grading of SOS/VOD in adults in the refined EBMT criteria are described as mild, moderate, severe and very severe/MOD (MOD, multiorgan dysfunction). They rely on time since clinical symptoms of SOS/VOD, bilirubin level (mg/dl), bilirubin kinetics, weight increase, and renal function (creatininemia). Do you find these criteria easy to use and useful in your practice in improving patient outcome? (Required.)

Yes

Other (please specify)

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18. At what point do you currently evaluate hepatic VOD severity in your clinical practice? (Required.)

Never

When VOD is suspected

As soon as diagnostic criteria are met

After differential diagnosis (when other complications are ruled out)

Other (please specify)

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19. At what level of severity do you prefer to start VOD/SOS therapy? (please select all that apply) (Required.)

Mild

Moderate

Severe

Very severe/MOD

I decide on a case per case basis (please specify)

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20. In your opinion, which additional members of the multidisciplinary team must be trained on hepatic VOD management following the refined criteria? (please select all that apply) (Required.)

Nurses

Hepatologists

Radiologists

Intensivists

Other (please specify)

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21. Would you like to make any additional comment/suggestion?

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22. To be eligible to win one of 20 complimentary registrations for the IACH Annual Meeting which will take place in Paris this coming October 2024, please provide your email.

An international survey to better understand the current incidence, severity, and management of VOD/SOS

Question Title

Question Title

* 1. What is your profession? (Required.)

Question Title

* 2. How old are you? (Required.)

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* 3. In what country do you practice? (Required.)

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* 4. How long have you been practicing? (Required.)

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* 5. Are you a member of, or aﬀiliated with any of the following national/regional societies/organizations? (Please select all that apply) (Required.)

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* 6. What is your primary focus in transplantation? (Please select all that apply) (Required.)

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* 7. On average, what percentage of your working time do you spend on the following activities (numbers must add up to 100%)? (Required.)

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* 8. Do you find it difficult to establish/make an early diagnosis of VOD/SOS in your practice? (Required.)

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* 9. Please explain your answer to the question above (Required.)

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* 10. What is the current incidence of liver VOD/SOS in your practice? (Required.)

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* 11. Do you see VOD/SOS outside allogeneic hematopoietic cell transplantation? (please select all that apply) (Required.)

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* 12. Which criteria do you use to establish the diagnosis of VOD/SOS? (please select all that apply) (Required.)

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* 13. The refined EBMT criteria (Bone Marrow Transplant. 2023 58:749-754. https://doi.org/10.1038/s41409-023-01992-8) introduced the concept of PROBABLE, CLASSICAL and PROVEN VOD/SOS. Do you feel this is useful in your practice to improve the diagnostic process of VOD/SOS? (Required.)

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* 14. How useful is the PROBABLE hepatic VOD diagnostic category for your clinical practice? (Required.)

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* 15. The unmodifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

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* 16. The modifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

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* 18. At what point do you currently evaluate hepatic VOD severity in your clinical practice? (Required.)

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* 19. At what level of severity do you prefer to start VOD/SOS therapy? (please select all that apply) (Required.)

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* 20. In your opinion, which additional members of the multidisciplinary team must be trained on hepatic VOD management following the refined criteria? (please select all that apply) (Required.)

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* 21. Would you like to make any additional comment/suggestion?

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* 22. To be eligible to win one of 20 complimentary registrations for the IACH Annual Meeting which will take place in Paris this coming October 2024, please provide your email.

1. What is your profession? (Required.)

2. How old are you? (Required.)

3. In what country do you practice? (Required.)

4. How long have you been practicing? (Required.)

5. Are you a member of, or aﬀiliated with any of the following national/regional societies/organizations? (Please select all that apply) (Required.)

6. What is your primary focus in transplantation? (Please select all that apply) (Required.)

7. On average, what percentage of your working time do you spend on the following activities (numbers must add up to 100%)? (Required.)

8. Do you find it difficult to establish/make an early diagnosis of VOD/SOS in your practice? (Required.)

9. Please explain your answer to the question above (Required.)

10. What is the current incidence of liver VOD/SOS in your practice? (Required.)

11. Do you see VOD/SOS outside allogeneic hematopoietic cell transplantation? (please select all that apply) (Required.)

12. Which criteria do you use to establish the diagnosis of VOD/SOS? (please select all that apply) (Required.)

13. The refined EBMT criteria (Bone Marrow Transplant. 2023 58:749-754. https://doi.org/10.1038/s41409-023-01992-8) introduced the concept of PROBABLE, CLASSICAL and PROVEN VOD/SOS. Do you feel this is useful in your practice to improve the diagnostic process of VOD/SOS? (Required.)

14. How useful is the PROBABLE hepatic VOD diagnostic category for your clinical practice? (Required.)

15. The unmodifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

16. The modifiable SOS/VOD risk factors for adults from the refined EBMT criteria are listed below. Please select the THREE most important risk factors that you consider in your practice? (Required.)

18. At what point do you currently evaluate hepatic VOD severity in your clinical practice? (Required.)

19. At what level of severity do you prefer to start VOD/SOS therapy? (please select all that apply) (Required.)

20. In your opinion, which additional members of the multidisciplinary team must be trained on hepatic VOD management following the refined criteria? (please select all that apply) (Required.)

21. Would you like to make any additional comment/suggestion?

22. To be eligible to win one of 20 complimentary registrations for the IACH Annual Meeting which will take place in Paris this coming October 2024, please provide your email.