Late-onset hepatic veno-occlusive disease after allografting: report of two cases with atypical clinical features successfully treated with defibrotide.

Alessia Castellino; Stefano Guidi; Chiara Dellacasa; Antonella Gozzini; Irene Donnini; Chiara Nozzoli; Sara Manetta; Semra Aydin; Luisa Giaccone; Moreno Festuccia; Lucia Brunello; Enrico Maffini; Benedetto Bruno; Ezio David; Alessandro Busca

doi:10.4084/mjhid.2018.001

	Late-onset hepatic veno-occlusive disease after allografting: report of two cases with atypical clinical features successfully treated with defibrotide. Alessia Castellino, Stefano Guidi, Chiara Dellacasa, Antonella Gozzini, Irene Donnini, Chiara Nozzoli, Sara Manetta, Semra Aydin, Luisa Giaccone, Moreno Festuccia, Lucia Brunello, Enrico Maffini, Benedetto Bruno, Ezio David, Alessandro Busca
DOI: https://doi.org/10.4084/mjhid.2018.001
Alessia Castellino Città della Salute e della Scienza University and Hospital, Hematology Unit, Turin, Italy, Italy \| acastellino@cittadellasalute.to.it Stefano Guidi SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze, Italy Chiara Dellacasa A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Antonella Gozzini SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze, Italy Irene Donnini SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze, Italy Chiara Nozzoli SODc Terapie Cellulari e Medicina Trasfusionale, AOU Careggi, Firenze, Italy Sara Manetta A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Semra Aydin A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, Ematologia, Torino, Italy, Italy Luisa Giaccone A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Moreno Festuccia A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Lucia Brunello A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Enrico Maffini A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Benedetto Bruno A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy Ezio David S.C. Anatomia Patologica 1, A.O.U. Città della Salute e della Scienza di Torino, Italy Alessandro Busca A.O.U. Città della Salute e della Scienza di Torino, Dipartimento di Oncologia, SSD Trapianto allogenico di cellule staminali, Torino, Italy, Italy
Abstract 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. Keywords Leukemia
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Submitted: 2017-08-02 20:40:18 Published: 2018-01-01 00:00:00 Search for citations in Google Scholar Related articles: Google Scholar References REFERENCES Carreras E, et al. Veno-occlusive disese of the liver after hemopoietic cell transplantation. Eur J Haematol 2000;64: 281-291. McDonald, G.B., Hinds, M.S., Fisher, L.D., et al. Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients. Annals of Internal Medicine 1993; 118, 255–267. Jones, R.J., Lee, K.S., Beschorner, W.E., et al. Venoocclusive disease of the liver following bone marrow transplantation. Transplantation, 1987;44, 778–783. Lee, J.L., Gooley, T., Bensinger, W., et al. Veno-occlusive disease of the liver after busulfan, melphalan, and thiotepa conditioning therapy: incidence, risk factors, and outcome. Biology of Blood and Marrow Transplantation, 1999;5, 306–315. Coppell JA, Richardson PG, Soiffer R, Martin PL, Kernan NA, Chen A et al. 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