Thielemans Naomi, De Beule Nathan, Van den Bergh Frans, Lefesvre Pierre, De Becker Ann
Department of Clinical Hematology, Universitair Ziekenhuis Brussel, Brussels, BEL.
Department of Interventional Radiology, Universitair Ziekenhuis Brussel, Brussels, BEL.
Cureus. 2024 Aug 24;16(8):e67682. doi: 10.7759/cureus.67682. eCollection 2024 Aug.
Sinusoidal obstruction syndrome (SOS) is a rare but potentially life-threatening complication, usually described in the setting of hematopoietic stem cell transplantation (HSCT). The very severe forms have a high mortality rate (>80%) and need fast recognition and urgent treatment. In this case report, we describe a unique and successful treatment strategy. We present a 27-year-old patient with newly diagnosed CD33+ acute myeloid leukemia (AML). She was treated with induction chemotherapy (7+3 regimen) and gemtuzumab ozogamicin (GO). In the absence of other major risk factors, she developed a very severe SOS with multiple organ failure. She was successfully treated with the urgent insertion of a transjugular intrahepatic portosystemic shunt (TIPS), defibrotide, and high-dose corticosteroids. This case of successful treatment for very severe SOS supports a combination strategy involving the immediate mechanical reduction of portal hypertension through TIPS and drug-mediated inhibition of microvascular thrombosis. Furthermore, this case shows the need for an improved prevention strategy, including the identification of additional risk factors and biomarkers.
窦性阻塞综合征（SOS）是一种罕见但可能危及生命的并发症，通常在造血干细胞移植（HSCT）的背景下被描述。非常严重的形式具有高死亡率（>80%），需要快速识别和紧急治疗。在本病例报告中，我们描述了一种独特且成功的治疗策略。我们介绍了一名27岁新诊断为CD33+急性髓系白血病（AML）的患者。她接受了诱导化疗（7+3方案）和吉妥珠单抗奥唑米星（GO）治疗。在没有其他主要危险因素的情况下，她发生了伴有多器官衰竭的非常严重的SOS。通过紧急插入经颈静脉肝内门体分流术（TIPS）、去纤苷和高剂量皮质类固醇，她得到了成功治疗。这例非常严重SOS的成功治疗病例支持一种联合策略，即通过TIPS立即机械性降低门静脉高压以及药物介导抑制微血管血栓形成。此外，该病例表明需要改进预防策略，包括识别额外的危险因素和生物标志物。
