Overview
New Diagnostic Criteria, Grading Classification, and Risk Factors for Sinusoidal Obstruction Syndrome (SOS)/Veno-Occlusive Disease (VOD)
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Sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease (VOD), is a life-threatening complication that can occur following hematopoietic stem cell transplant (HSCT) and is also associated with some anti-cancer drugs, such as gemtuzumab ozogamicin and inotuzumab ozogamicin. It can occur in up to 15% of high-risk patients after HSCT, and almost half of cases present with aggressive disease leading to multiorgan failure with a mortality rate exceeding 80%.
Early diagnosis and severity grading are paramount to reducing mortality, but early identification and severity assessment of SOS/VOD is often difficult secondary to the lack of sensitivity and specificity with traditional diagnostic criteria. Moreover, differing diagnostic criteria for VOD, especially between adults and children, can lead to variable reported incidence and possibly, under recognition and underdiagnosis in patients.
Newer diagnostic and grading criteria have been developed that help overcome these limitations, incorporating the latest insights with SOS/VOD pathophysiology, but clinicians need support in translating these new criteria into routine clinical practice. This digital FAQ, designed with your questions in mind, offers succinct and relevant information for clinicians on an array of questions to support this translation and enhance their diagnostic-therapeutic paradigms for SOS/VOD.
This activity is intended for hematologists, oncologists, bone marrow transplant physicians, intensivists, hepatologists, oncology nurses and the multidisciplinary healthcare team who are involved in the care of patients with SOS.
Sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease (VOD), is a life-threatening complication that can occur following hematopoietic stem cell transplant (HSCT) and is also associated with some anti-cancer drugs, such as gemtuzumab ozogamicin and inotuzumab ozogamicin. SOS/VOD can occur in up to 15% of high-risk patients after HSCT, and almost half of cases present with aggressive disease leading to multiorgan failure with a mortality exceeding 80%. Early diagnosis and severity grading are paramount to reducing mortality so clinicians must understand how to mitigate and manage risk of SOS/VOD. Conventional criteria carry the risk of underdiagnosing SOS/VOD, but newer diagnostic and grading criteria have been developed to help overcome these limitations and incorporate the latest insights with SOS/VOD pathophysiology. Clinicians also require a clear understanding of available pharmacotherapy and dosing because delay of administration for SOS/VOD with multiorgan dysfunction results in an increased risk of mortality. To effectively translate expert guidance to practice and build clinician confidence in making an accurate diagnosis and optimizing therapeutic benefit through management of SOS/VOD, clinicians would benefit from peer-to-peer exchanges to model best practices and discuss practical strategies to achieve goals.
Upon completion of this activity, learners will be able to:
• Describe the pathophysiology of SOS/VOD
• Illustrate best practices to facilitate an early diagnosis of SOS/VOD
• Identify past, present, and new risk factors of SOS/VOD
• Outline modern grading classification for SOS/VOD
• Apply clinical evidence to optimize therapeutic decision-making in patients with SOS/VOD
• Describe the pathophysiology of SOS/VOD
• Illustrate best practices to facilitate an early diagnosis of SOS/VOD
• Identify past, present, and new risk factors of SOS/VOD
• Outline modern grading classification for SOS/VOD
• Apply clinical evidence to optimize therapeutic decision-making in patients with SOS/VOD
Provided by the Academy for Continued Healthcare Learning (ACHL).
Supported by an educational grant from Jazz Pharmaceuticals.
Mitchell S. Cairo, MD
Chief, Pediatric Hematology, Oncology, and Stem Cell Transplantation
Director, Children and Adolescent Cancer and Blood Diseases Center
Director, WMC Cancer Center
Medical and Scientific Director, WMC Cellular and Tissue Engineering Laboratory
Medical Director, WMC Hematotherapy Program
Vice Chairman of Pediatrics
Professor of Pediatrics, Medicine, Pathology, Microbiology
& Immunology and Cell Biology & Anatomy
Maria Fareri Children’s Hospital
Westchester Medical Center (WMC)
New York Medical College
New York, NY
Chief, Pediatric Hematology, Oncology, and Stem Cell Transplantation
Director, Children and Adolescent Cancer and Blood Diseases Center
Director, WMC Cancer Center
Medical and Scientific Director, WMC Cellular and Tissue Engineering Laboratory
Medical Director, WMC Hematotherapy Program
Vice Chairman of Pediatrics
Professor of Pediatrics, Medicine, Pathology, Microbiology
& Immunology and Cell Biology & Anatomy
Maria Fareri Children’s Hospital
Westchester Medical Center (WMC)
New York Medical College
New York, NY
Kenneth Cooke, MD
Director, Pediatric Blood and Marrow Transplantation Program
Herman and Walter Samuelson Professor of Oncology
Sidney Kimmel Comprehensive Cancer Center
Johns Hopkins University School of Medicine
Baltimore, MD
The Academy for Continued Healthcare Learning (ACHL) requires that the faculty participating in an accredited continuing education activity disclose all affiliations or other financial relationships within 24 months (1) with the manufacturers of any commercial product(s) and/or provider(s) of commercial services discussed in an educational presentation and (2) with all ineligible companies. All relevant financial relationships have been mitigated prior to this activity.
The following financial relationships have been provided:
Mitchell S. Cairo, MD (Chair)
Sources of Funding for Research: Jazz Pharmaceuticals
Consulting Agreements: Jazz Pharmaceuticals
Speakers' Bureau: Jazz Pharmaceuticals
Kenneth Cooke, MD (Chair)
Consultant (occasional): Jazz Pharmaceuticals
Advisory board member: Jazz Pharmaceuticals
Honorarium recipient: Jazz Pharmaceuticals
Research support recipient: Jazz Pharmaceuticals
ACHL staff members and others involved with the planning, development, and review of the content for this activity have no relevant affiliations or financial relationships to disclose.
The content for this activity was developed independently of any ineligible company. All materials are included with permission. The opinions expressed are those of the faculty and are not to be construed as those of the publisher or grantor(s).
This educational activity was planned and produced in accordance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education. Recommendations involving clinical medicine in a continuing medical education (CME/CE) activity must be based on evidence that is accepted within the profession of medicine as adequate justification for their indications and contraindications in the care of patients. All scientific research referred to, reported, or used in CME/CE in support or justification of a patient care recommendation must conform to the generally accepted standards of experimental design, data collection, and analysis.
This CME/CE activity might describe the off-label, investigational, or experimental use of medications and/or devices that may exceed their FDA-approved labeling. Physicians should consult the current manufacturers’ prescribing information for these products. ACHL requires the speaker to disclose that a product is not labeled for the use under discussion.
Discussion of scientific information on unapproved uses (SIUU), off-label, investigational, or experimental drug/device use: Treatment of SOS/VOD with defibrotide in patients without renal or pulmonary dysfunction. Prophylactic use of ursodiol and defibrotide for SOS/VOD.
This activity will take approximately 60 minutes to complete. To receive credit, learners are required to complete the pretest, view the online activity, and complete the posttest and evaluation. To receive credit, 60% must be achieved on the posttest. A certificate will be immediately available. There is no fee to participate in the activity or for the generation of the certificate.
The Academy for Continued Healthcare Learning is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.The Academy for Continued Healthcare Learning designates this enduring material for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Physician assistants, nurse practitioners, and nurses may participate in this educational activity and earn a certificate of completion as AAPA, AANP, and ANCC accept AMA PRA Category 1 Credits™ through their reciprocity agreements.
