Escobar Gil Tomas, Varatharaj Sushrruti, Rosenberg Stephanie K, Aguilar Gicel J, Santistevan David, Azevedo Keith, Mindiola Romero Andres E
Internal Medicine, University of New Mexico School of Medicine, Albuquerque, USA.
Emergency Medicine, University of New Mexico School of Medicine, Albuquerque, USA.
Cureus. 2025 Mar 5;17(3):e80078. doi: 10.7759/cureus.80078. eCollection 2025 Mar.
We present the case of a 41-year-old female with chronic myeloid leukemia (CML) in blast crisis who underwent haploidentical allogeneic stem cell transplantation. Her post-transplant course was complicated by neutropenic sepsis and mucositis, followed by the development of sinusoidal obstruction syndrome (SOS) on post-transplant day (POD) 10, evidenced by rising hyperbilirubinemia (peak 22.2 mg/dL) and significant weight gain primarily due to fluid retention. Imaging confirmed hepatosplenomegaly and portal vein dilation, leading to the SOS diagnosis. Defibrotide was initiated on POD 16, leading to a progressive decline in bilirubin levels (from 22.2 mg/dL to 2.4 mg/dL by POD 22), suggesting a therapeutic response. However, thrombocytopenia and gastrointestinal hemorrhage necessitated dose interruptions. Supportive care included fluid management, albumin infusions, and diuresis, but hepatorenal syndrome developed, requiring continuous renal replacement therapy (CRRT). On POD 27, she developed acute hypoxic respiratory failure, requiring a high-flow nasal cannula and later vasopressor support for worsening hemodynamic instability. Despite intensified critical care measures, including broad-spectrum antimicrobials and transfusion support, her condition deteriorated, leading to progressive multiorgan failure and transition to comfort care on POD 34. This case highlights the diagnostic and therapeutic challenges of severe post-transplant SOS, emphasizing the need for early intervention, a tailored risk-benefit assessment of defibrotide, and multidisciplinary critical care strategies for high-risk patients.
我们报告了一例41岁处于急变期的慢性髓性白血病（CML）女性患者，她接受了单倍体相合异基因干细胞移植。她的移植后病程因中性粒细胞减少性脓毒症和粘膜炎而复杂化，随后在移植后第10天（POD 10）出现了肝窦阻塞综合征（SOS），表现为高胆红素血症升高（峰值22.2mg/dL）以及主要由于液体潴留导致的显著体重增加。影像学检查证实了肝脾肿大和门静脉扩张，从而确诊为SOS。在POD 16开始使用去纤苷，胆红素水平逐渐下降（到POD 22时从22.2mg/dL降至2.4mg/dL），提示有治疗反应。然而，血小板减少症和胃肠道出血需要中断用药剂量。支持性治疗包括液体管理、输注白蛋白和利尿，但出现了肝肾综合征，需要持续肾脏替代治疗（CRRT）。在POD 27，她出现了急性低氧性呼吸衰竭，需要高流量鼻导管吸氧，后来因血流动力学不稳定恶化需要血管活性药物支持。尽管加强了重症监护措施，包括使用广谱抗菌药物和输血支持，她的病情仍恶化，导致进行性多器官功能衰竭，并在POD 34转为舒适护理。该病例突出了移植后严重SOS的诊断和治疗挑战，强调了早期干预的必要性、对去纤苷进行个性化风险效益评估以及针对高危患者的多学科重症监护策略。
