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Post-transplant endothelial disorder after hematopoietic SCT: a blinded autopsy study

Bone Marrow Transplantation volume 47, pages 1243–1245 (2012)Cite this article

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Vascular endothelial injury is a serious complication and often leads to organ dysfunction after hematopoietic SCT (HSCT). Multiple factors, including acute GVHD, calcineurin inhibitors and infections, can contribute to the endothelial damage in post-transplant patients.1, 2 Because of similarities in histology, such vascular injury has been considered a member of thrombotic microangiopathy (TMA), including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome . However, unlike other TMAs, post-transplant endothelial damage has rarely been associated with severe deficiency of ADAMTS13 and does not respond well to plasma exchange.3 Thus, the ‘post-transplant endothelial disorder (PTED)’ has distinct features, and its pathophysiology remains uncertain.

Six (40%) of the 15 patients showed histologic evidence of PTED in small intestine (patient (pt.) 1–5) or in kidney (pt. 1, 2, 6). There were 5 (33%) additional cases showing mild vascular changes only in kidney, and these cases were diagnosed as ‘possible’ PTED. Four (27%) patients showed no histologic evidence of PTED. Among the six patients with definite PTED, 4 (67%) patients were clinically diagnosed with TMA, but the other two patients (Pt. 2 and 5) were not. TMA was diagnosed by clinical findings including intravascular hemolysis associated with the detection of increased fragmented erythrocytes. None of the five patients with possible PTED were diagnosed with TMA after HSCT (Table 1). Thus, some post-transplant patients did not show typical manifestations of TMA even with severe vascular injury. We speculate that severity and the primary site (intestine or kidney) of vascular damage affect clinical presentations of PTED patients. Larger studies will be needed to reveal the link between vascular injury and clinical manifestations in post-transplant patients.

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References

  1. Batts ED, Lazarus HM . Diagnosis and treatment of transplantation-associated thrombotic microangiopathy: real progress or are we still waiting? Bone Marrow Transplant 2007; 40: 709–719.

    Article  CAS  PubMed  Google Scholar 

  2. Laskin BL, Goebel J, Davies SM, Jodele S . Small vessels, big trouble in the kidneys and beyond: hematopoietic stem cell transplant associated-thrombotic microangiopathy. Blood 2011; 118: 1452–1462.

    Article  CAS  PubMed  Google Scholar 

  3. Vesely SK, George JN, Lammle B, Studt JD, Alberio L, El-Harake MA et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003; 102: 60–68.

    Article  CAS  PubMed  Google Scholar 

  4. Siami K, Kojouri K, Swisher KK, Selby GB, George JN, Laszik ZG . Thrombotic microangiopathy after allogeneic hematopoietic stem cell transplantation: an autopsy study. Transplantation 2008; 85: 22–28.

    Article  PubMed  Google Scholar 

  5. Changsirikulchai S, Myerson D, Guthrie KA, McDonald GB, Alpers CE, Hingorani SR . Renal thrombotic microangiopathy after hematopoietic cell transplant: role of GVHD in pathogenesis. Clin J Am Soc Nephrol 2009; 4: 345–353.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Sale GE, Shulman HM . The Pathology of Bone Marrow Transplantation, vol.ix Masson Pub. USA: New York, 1984, p 265.

    Google Scholar 

  7. Biedermann BC . Vascular endothelium and graft-versus-host disease. Best Pract Res Clin Haematol 2008; 21: 129–138.

    Article  CAS  PubMed  Google Scholar 

  8. Rosenthal J, Pawlowska A, Bolotin E, Cervantes C, Maroongroge S, Thomas SH et al. Transplant-associated thrombotic microangiopathy in pediatric patients treated with sirolimus and tacrolimus. Pediatr Blood Cancer 2010; 57: 142–146.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Goyama S, Kanda Y, Nannya Y, Ogawa S, Asano-Moki Y, Ogawa N et al. Clinical significance of peripheral blood erythroblastosis after hematopoietic stem cell transplantation. Leuk Lymphoma 2004; 45: 2439–2443.

    Article  PubMed  Google Scholar 

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Acknowledgements

KT conceived the concept of post-transplant endothelial disorder.

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Author notes

  1. S Goyama

    Present address: Current address: Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45242, USA,

  2. K Takeuchi

    Present address: Current address: Pathology Project for Molecular Targets, The Cancer Institute, Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto, Tokyo 135-8550, Japan,

  3. Y Kanda

    Present address: Current address: Division of Hematology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama-shi, Saitama 330-8503, Japan,

  4. S Chiba

    Present address: Current address: Department of Clinical and Experimental Hematology, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan,

Authors and Affiliations

  1. Department of Hematology and Oncology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan

    S Goyama, Y Kanda, Y Nannya, S Chiba & M Kurokawa

  2. Department of Pathology, the University of Tokyo Hospital, Tokyo, Japan

    K Takeuchi & M Fukayama

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  1. S Goyama
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Correspondence to M Kurokawa.

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Goyama, S., Takeuchi, K., Kanda, Y. et al. Post-transplant endothelial disorder after hematopoietic SCT: a blinded autopsy study. Bone Marrow Transplant 47, 1243–1245 (2012). https://doi.org/10.1038/bmt.2011.243

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