Castellino Alessia, Guidi Stefano, Dellacasa Chiara Maria, Gozzini Antonella, Donnini Irene, Nozzoli Chiara, Manetta Sara, Aydin Semra, Giaccone Luisa, Festuccia Moreno, Brunello Lucia, Maffini Enrico, Bruno Benedetto, David Ezio, Busca Alessandro
A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, Ematologia, Torino, Italy.
SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze.
Mediterr J Hematol Infect Dis. 2018 Jan 1;10(1):e2018001. doi: 10.4084/MJHID.2018.001. eCollection 2018.
Hepatic Veno-Occlusive Disease (VOD) is a potentially severe complication of hematopoietic stem cell transplantation (HSCT). Here we report two patients receiving an allogeneic HSCT who developed late onset VOD with atypical clinical features. The two patients presented with only few risk factors, namely, advanced acute leukemia, a myeloablative busulphan-containing regimen and received grafts from an unrelated donor. The first patient did not experience painful hepatomegaly and weight gain and both patients showed only a mild elevation in total serum bilirubin level. Most importantly, the two patients developed clinical signs beyond day 21 post-HSCT. Hepatic transjugular biopsy confirmed the diagnosis of VOD. Intravenous defibrotide was promptly started leading to a marked clinical improvement. Based on our experience, liver biopsy may represent a useful diagnostic tool when the clinical features of VOD are ambiguous. Early therapeutic intervention with defibrotide represents a crucial issue for the successful outcome of patients with VOD.
肝静脉闭塞病（VOD）是造血干细胞移植（HSCT）的一种潜在严重并发症。在此我们报告两名接受异基因HSCT的患者，他们发生了具有非典型临床特征的迟发性VOD。这两名患者仅有少数危险因素，即晚期急性白血病、含白消安的清髓方案以及接受来自无关供者的移植物。第一名患者未出现肝肿大疼痛和体重增加，且两名患者血清总胆红素水平仅轻度升高。最重要的是，这两名患者在HSCT后第21天之后出现临床症状。经颈静脉肝活检确诊为VOD。立即开始静脉输注去纤苷，临床症状显著改善。根据我们的经验，当VOD的临床特征不明确时，肝活检可能是一种有用的诊断工具。去纤苷的早期治疗干预是VOD患者获得成功预后的关键问题。
