Alessia Castellino1, Stefano Guidi2, Chiara Maria Dellacasa3, Antonella Gozzini2, Irene Donnini2, Chiara Nozzoli2, Sara Manetta3, Semra Aydin1, Luisa Giaccone3, Moreno Festuccia3, Lucia Brunello3, Enrico Maffini3, Benedetto Bruno3, Ezio David4 and Alessandro Busca3.
1 A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, Ematologia, Torino, Italy.
2 SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze.
3 A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy.
4 S.C. Anatomia Patologica 1, A.O.U. Città della Salute e della Scienza di Torino.
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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.
|Table 1. Criteria for definition of Late-Onset VOD (according to “The new classification from the European Society for Blood and Marrow Transplantation”, BMT 2016).|
|Figure 1. Images of hepatic transjugular biopsy: in the middle, the red arrows showed the expansion of hepatic sinusoid spaces, on the right the figure showed a centrilobular vein|
|Figure 2. Diagnostic interventions with liver function profile from clinical onset of VOD until resolution, and treatment of VOD in case 1.|
|Figure 3. Diagnostic interventions with liver function profile from clinical onset of VOD until resolution, and treatment of VOD in case 2.|