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
  • 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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Published: 2018-01-01 00:00:00
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