O IMPACTO DO DEFIBROTIDE NA SÍNDROME DE OBSTRUÇÃO SINUSOIDAL NO PÓS TRANSPLANTE DE MEDULA ÓSSEA: REVISÃO INTEGRATIVA DA LITERATURA

• DOI: https://doi.org/10.22533/at.ed.3232524049

• Palavras-chave: transplante de medula óssea; doença veno-obstrusiva hepática; medicamentos inovadores.

• Keywords: bone marrow transplant; veno-occlusive liver disease; innovative drugs.

• Abstract: Sinusoidal Obstruction Syndrome (SOS) also represents a significant complication of hematopoietic stem cell transplantation (HSCT), with incidence rates of up to 13%. The characteristic clinical signs and symptoms of SOS include weight gain, hyperbilirubinemia, ascites, and painful hepatomegaly. Defibrotide is the only registered drug for the treatment of moderate/severe SOS; it is a mixture of polidesoxyribonucleotides derived from porcine intestinal mucosa. Its mechanism of action is not yet fully understood. Thus, the objective of this study is to identify in the literature the effects of SOS and its impact on morbidity and mortality in patients diagnosed post-HSCT. This study is an integrative review. The bibliographic survey was conducted through electronic search in the following databases: Latin American and Caribbean Center on Health Sciences Information (LILACS), Cochrane Library, PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and SCOPUS. Regarding inclusion criteria, full-text articles available electronically in Portuguese, English, and Spanish, from January to October 2024, were selected if they addressed the topic in the title, abstract, or descriptors. Using the proposed methodology and search strategy, 460 articles were found in the selected databases. After the selection and inclusion steps, 11 articles were eligible for this integrative review. SOS has a variable incidence, influenced by factors such as the type of transplant and the conditioning regimen. Early diagnosis is essential, and the European Group for Blood and Marrow Transplantation (EBMT) 2023 criteria highlight elevated bilirubin as a key indicator. Treatment with defibrotide has shown effectiveness, particularly when started early, improving survival rates. Prophylaxis with defibrotide also reduces the incidence of SOS, being cost-effective, especially in high-risk patients. These findings reinforce the importance of early diagnosis and appropriate therapies to improve clinical outcomes and reduce the impacts of SOS.