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Castellino A, Guidi S, Dellacasa CM, et al. Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. Mediterr J Hematol Infect Dis. 2018;10(1):e2018001doi: 10.4084/MJHID.2018.001.
Castellino, A., Guidi, S., Dellacasa, C. M., Gozzini, A., Donnini, I., Nozzoli, C., Manetta, S., Aydin, S., Giaccone, L., Festuccia, M., Brunello, L., Maffini, E., Bruno, B., David, E., & Busca, A. (2018). Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. Mediterranean journal of hematology and infectious diseases, 10(1), e2018001. https://doi.org/10.4084/MJHID.2018.001
Castellino, Alessia, et al. "Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide." Mediterranean journal of hematology and infectious diseases vol. 10,1 (2018): e2018001. doi: https://doi.org/10.4084/MJHID.2018.001
Castellino A, Guidi S, Dellacasa CM, Gozzini A, Donnini I, Nozzoli C, Manetta S, Aydin S, Giaccone L, Festuccia M, Brunello L, Maffini E, Bruno B, David E, Busca A. Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide. Mediterr J Hematol Infect Dis. 2018 Jan 01;10(1):e2018001. doi: 10.4084/MJHID.2018.001. eCollection 2018. PMID: 29326798; PMCID: PMC5760078.
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Mediterr J Hematol Infect Dis. 2018 Jan 01;10(1):e2018001. doi: 10.4084/MJHID.2018.001. eCollection 2018.

Late-Onset Hepatic Veno-Occlusive Disease after Allografting: Report of Two Cases with Atypical Clinical Features Successfully Treated with Defibrotide.

Mediterranean journal of hematology and infectious diseases

Alessia Castellino, Stefano Guidi, Chiara Maria 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

Affiliations

  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.

PMID: 29326798 PMCID: PMC5760078 DOI: 10.4084/MJHID.2018.001

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: Allogeneic Hematopietic stem cell transplantation; Leukemia; VOD; Veno-occlusive disease

Conflict of interest statement

Competing interests: The authors have declared that no competing interests exist.

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