Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Aug 13;3(15):2424-2435.
doi: 10.1182/bloodadvances.2019000143.

Allogeneic reactivity-mediated endothelial cell complications after HSCT: a plea for consensual definitions

Simona Pagliuca  1   2 , David Michonneau  1   2 , Flore Sicre de Fontbrune  1 , Aurélien Sutra Del Galy  1   2 , Aliénor Xhaard  1 , Marie Robin  1 , Régis Peffault de Latour  1   2 , Gérard Socie  1   2
Affiliations
Review

Allogeneic reactivity-mediated endothelial cell complications after HSCT: a plea for consensual definitions

Simona Pagliuca et al. Blood Adv. .

Abstract

Endothelial cell (EC) activation has been suspected of triggering a group of rare and dismal complications that can occur after allogeneic hematopoietic stem cell transplantation (HSCT). Capillary leak syndrome, engraftment syndrome, transplant-associated microangiopathy, diffuse alveolar hemorrhage, and idiopathic pneumonia syndrome are the main nosological entities. Post-HSCT endotheliitis can be triggered by chemotherapy, infections, and calcineurin inhibitors, but allogeneic reactivity is claimed to be the common denominator. Endothelial damages are thought to activate several deleterious pathways (proapoptotic, procoagulant, proinflammatory) and can lead to multiorgan failure; however, clinical manifestations of each syndrome overlap, and their relationship with graft-versus-host disease could be minimal. The lack of well-defined diagnostic criteria does not allow for a clear-cut comparison in the current literature. Therapeutic efforts have been made to intercept the pathogenic mechanisms leading to EC dysfunction, but remission rates and survival remain mostly unsatisfactory. In this article, we have reviewed the incidence, clinical features, and treatment approaches of EC activation syndromes, and we plead for the development of internationally accepted standard definitions.

PubMed Disclaimer

Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
Spectrum and pathophysiology of post-HSCT endothelial complications. The Venn diagram highlights the overlap of endothelial syndromes, both diagnostically and pathophysiologically, with GVHD. Many clinical and pathophysiological features of GVHD parallel those of other EC activation syndromes in which a single organ is targeted. Here, atherosclerosis and several delayed forms of IPS have been proposed as late endothelial dysfunction syndromes overlapping with chronic GVHD. DAH, diffuse alveolar hemorrhage; PERDS, peri-engraftment respiratory distress syndrome.
Figure 2.
Figure 2.
Proposed management of endothelial syndromes.
See this image and copyright information in PMC

References

    1. Inamoto Y, Lee SJ. Late effects of blood and marrow transplantation. Haematologica. 2017;102(4):614-625. - PMC - PubMed
    1. Pasquini MC, Wang Z, Horowitz MM, Gale RP. 2010 report from the Center for International Blood and Marrow Transplant Research (CIBMTR): current uses and outcomes of hematopoietic cell transplants for blood and bone marrow disorders. Clin Transpl. 2010;2010:87-105. - PubMed
    1. Carreras E, Diaz-Ricart M. The role of the endothelium in the short-term complications of hematopoietic SCT. Bone Marrow Transplant. 2011;46(12):1495-1502. - PubMed
    1. Spitzer TR. Engraftment syndrome following hematopoietic stem cell transplantation. Bone Marrow Transplant. 2001;27(9):893-898. - PubMed
    1. Ruutu T, Barosi G, Benjamin RJ, et al. ; European LeukemiaNet . Diagnostic criteria for hematopoietic stem cell transplant-associated microangiopathy: results of a consensus process by an International Working Group. Haematologica. 2007;92(1):95-100. - PubMed

MeSH terms