Evans Syndrome following T Cell-repleted Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome: Successful Response to High-dose Corticosteroid

Young-Ho Lee; Young-Dae Kim; Yeong-Myung Kim

doi:10.5045/kjh.2006.41.3.204

Journal List > Korean J Hematol > v.41(3) > 1032676

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Lee, Kim, and Kim: Evans Syndrome following T Cell-repleted Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome: Successful Response to High-dose Corticosteroid

Case Report

Korean J Hematol 2006;41(3):204-207.

Published online: 19 September 2006

DOI: https://doi.org/10.5045/kjh.2006.41.3.204

Evans Syndrome following T Cell-repleted Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome: Successful Response to High-dose Corticosteroid

Young-Ho Lee, M.D.^1,, Young-Dae Kim, M.D.², Yeong-Myung Kim, M.D.³

¹Department of Pediatrics Hanyang University Medical Center, Seoul

²Inje University Paik Hospital, Seoul

³Dong-A University Medical Center, Busan, Korea

Correspondence to: Young-Ho Lee, M.D. Department of Pediatrics, Hanyang University Medical Center 17, Haengdang-dong, Seongdong-gu, Seoul 133-792, Korea Tel: +82-2-2290-8383, Fax: +82-2-2297-2380 E-mail: cord@hanyang.ac.kr

Received 2 June 2006 Revised 19 July 2006 Accepted 24 July 2006

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Evans syndrome after hematopoietic stem cell transplantation rarely occurs and this condition is refractory to treatment, particularly in childhood. We experienced a case of Evans syndrome following unmanipulated unrelated bone marrow transplantation. Evans syndrome developed at early post-transplantation period (day +42) and it responded very well to high-dose corticosteroid. Autoimmune cytopenia should be considered for post-engraftment cytopenias and high-dose corticosteroid therapy might be considered as a treatment of the early onset type of autoimmune cytopenia that can occur after hematopoietic stem cell transplantation.

Go to :

Keywords: Evans syndrome, T cell repleted bone marrow transplantation, High-dose corticosteroid

REFERENCES

1). Drobyski WR., Potluri K., Sauer D., Gottschall JL. Autoimmune hemolytic anemia following T cell-depleted allogeneic bone marrow transplantation. Bone Marrow Transplant. 1996. 17:1093–9.

2). Pratt G., Kinsey SE. Remission of severe, intractable autoimmune haemolytic anaemia following matched unrelated donor transplantation. Bone Marrow Transplant. 2001. 28:791–3.

3). De Lord C., Marsh JC., Smith JG., Singer CR., Gordon-Smith EC. Fatal autoimmune pancytopenia following bone marrow transplantation for aplastic anaemia. Bone Marrow Transplant. 1996. 18:237–9.

4). Cwynarski K., Goulding R., Pocok C, et al. Immune haemolytic anaemia following T cell-depleted allogeneic bone marrow transplantation for chronic myeloid leukaemia: association with leukaemic relapse and treatment with donor lymphocyte infusions. Bone Marrow Transplant. 2001. 28:581–6.

5). Jillella AP., Kallab AM., Kutlar A. Autoimmune thrombocytopenia following autologous hematopoietic cell transplantation: review of literature and treatment options. Bone Marrow Transplant. 2000. 26:925–7.

6). Wahid FS., Cheong SK., Sivagengei K. Autoimmune thrombocytopenia and neutropenia after autologous peripheral blood stem cell transplantation. Acta Haematol. 2002. 107:237–38.

7). Evans RS., Takahashi K., Duane RT., Payne R., Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951. 87:48–65.

8). Pui CH., Wilimas J., Wang W. Evans syndrome in childhood. J Pediatr. 1980. 97:754–8.

9). Wang WC. Evans syndrome in childhood: Pathophysiology, clinical course and treatment. Am J Pediatr Hematol Oncol. 1988. 10:330–8.

10). Wang W., Herrod H., Pui CH., Presbury G., Wilimas J. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983. 15:381–90.

11). Dovat S., Roberts RL., Wakim M., Stiehm ER., Feig SA. Immune thrombocytopenia after umbilical cord progenitor cell transplant: response to vincristine. Bone Marrow Transplant. 1999. 24:321–3.

12). Winiarski J., Ringdén O., Remberger M., Dalianis T., Ljungman P., Borgström B. Bone marrow transplantation in children using unrelated donors. Acta Paediatr. 1996. 85:327–35.

13). Chen FE., Owen I., Savage D, et al. Late onset hemolysis and red cell autoimmunisation after allogeneic bone marrow transplant. Bone Marrow Transplant. 1997. 19:491–5.

14). Keung YK., Cobos E., Bolanos-Meade J., Issarachai S., Brideau A., Morgan D. Evans syndrome after autologous bone marrow for recurrent Hodgkin's disease. Bone Marrow Transplant. 1997. 20:1099–101.

15). Bashey A., Owen I., Lucas GF, et al. Late onset immune pancytopenia following bone marrow transplantation. Br J Haematol. 1991. 78:268–74.

Go to :

Fig. 1

Laboratory parameters before and after onset of Evans syndrome following unrelated bone marrow transplantation. Abbreviations: PLT, platelet; Hb, hemoglobin.

undefined

TOOLS

Similar articles