Late-Onset Veno-Occlusive Disease (VOD) Post-Haploidentical Transplant: A Diagnostic Challenge Requiring Liver Biopsy– A Case Report
Main Article Content
Keywords
Haploidentical HSCT, sinusoidal obstruction syndrome
Abstract
Abstract
Background: Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially fatal complication post-hematopoietic stem cell transplantation (HSCT), classically presenting within the first 3 weeks. However, late-onset VOD, often presenting beyond day +21, may lack the classical hallmarks, making diagnosis particularly challenging.
Case Presentation: We report a 41-year-old female with adverse-risk acute myeloid leukemia (AML) who underwent haploidentical HSCT in first complete remission. On day +35, she developed hemorrhagic cystitis due to BK polyomavirus and CMV viremia, followed by progressive abdominal distension, hepatomegaly, ascites, and mild hyperbilirubinemia. Initial imaging and laboratory investigations were non-specific. A diagnostic transjugular liver biopsy performed on day +52 revealed classical features of SOS with zone 3 perisinusoidal fibrosis. She was managed conservatively with complete clinical resolution.
Conclusion: This case highlights the diagnostic complexity of late-onset VOD and reinforces the indispensable role of liver biopsy when conventional criteria are inconclusive.
Downloads
Abstract 50
PDF Downloads 16
Html Downloads 8
References
2. Castellino A, Guidi S, Dellacasa CM, Gozzini A, Donnini I, Nozzoli C, et al. Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. Mediterr J Hematol Infect Dis. 2018;10(1):e2018001. doi:10.4084/MJHID.2018.001. PMID: 29326798
3. Corbacioglu S, Jabbour EJ, Mohty M. Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome. Biol Blood Marrow Transplant. 2019;25(7):1271–80. doi: 10.1016/j.bbmt.2019.02.018
4. Coppell JA, Richardson PG, Soiffer R, Martin PL, Kernan NA, Chen A, et al. Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome. Biol Blood Marrow Transplant. 2010;16(2):157–68. doi: 10.1016/j.bbmt.2009.08.024. PMID: 19766729
5. Corbacioglu S, Carreras E, Ansari M, Balduzzi A, Cesaro S, Dalle JH, et al. Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European Society for Blood and Marrow Transplantation. Br J Haematol. 2018;180(6):931–8. doi: 10.1111/bjh.15058
6. Fan CQ, Crawford JM. Sinusoidal obstruction syndrome (hepatic veno-occlusive disease). J Clin Exp Hepatol. 2014;4(4):332–46. doi:10.1016/j.jceh.2014.10.002
7. Richardson PG, Smith AR, Bleakley M, Blazar BR, Schuening FG, Parikh S, et al. Defibrotide for the treatment of hepatic veno-occlusive disease. Biol Blood Marrow Transplant. 2010;16(2):199–206. doi: 10.1016/j.bbmt.2009.09.017
8. Ruggiu M, Bedossa P, Rautou PE, Bertheau P, Plessier A, de Latour RP, et al. Utility and safety of liver biopsy in patients with undetermined liver blood test anomalies after allogeneic hematopoietic stem cell transplantation: a monocentric retrospective cohort study. Biol Blood Marrow Transplant. 2018;24(12):2523–31. doi: 10.1016/j.bbmt.2018.07.037
Article Sidebar
Article Details
How to Cite
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Transfer of Copyright and Permission to Reproduce Parts of Published Papers. Authors will grant copyright of their articles to the Institute of Hematology, Catholic University, Rome . No formal permission will be required to reproduce parts (tables or illustrations) of published papers, provided the source is quoted appropriately and reproduction has no commercial intent. Reproductions with commercial intent will require written permission and payment of royalties.