Abstract
A 10-year-old girl who had been treated as an outpatient for neurofibromatosis type 1 was admitted with fever, 10% weight loss, and abdominal pain that had persisted for 1 month. The clinical manifestations and the results of endoscopy led to the diagnosis of intestinal Behçet disease. Blood cell dysplasia appeared during the hospitalization. The patient subsequently developed blast cells, and the diagnosis of myelodysplastic syndrome (MDS) was made. Behçet disease was first controlled with steroids and diet followed by chemotherapy and cord blood stem cell transplantation (SCT). Both the MDS and Behçet disease went into remission after transplantation. The use of hematopoietic SCT has the potential not only to cure but also to help explicate the mechanism of Behçet disease.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Chajek T, Fainaru M. Behçet’s disease: report of 41 cases and review of the literature.Medicine. 1975;54:179–195.
Oen K. Comparative epidemiology of the rheumatic diseases in children.Curr Opin Rheumatol. 2000;12:410–414.
Gavin CH, Edward VL, Ayalew T, et al. Concurrent inflammatory bowel disease and myelodysplastic syndromes.Inflamm Bowel Dis. 1999;5:98–103.
Yano K, Eguchi K, Migita K, et al. Behçet’s disease complicated myelodysplastic syndrome: a report of two cases and review of the literature.Clin Rheumatol. 1996;15:91–93.
Wechsler B, Davatchi F, Mizushima K, et al. Criteria for diagnosis of Behçet’s disease.Lancet. 1990;335:1078–1080.
Saylan T, Mat C, Fresko I. Behçet’s disease in the Middle East.Clin Dermatol. 1999;17:209–223.
Isabelle KP, Sebabattin Y, Sultan AB,et al. Clinical features of Behçet’s disease in children: an international collaborative study of 86 cases.J Pediatr. 1998;132:721–725.
Fujikawa S, Suemitsu T. Behçet’s disease in children: a nationwide retrospective survey in Japan.Acta Paediatr Jpn. 1997;39:285–289.
Suh YL, Sung RH, Chi JG, et al. Intestinal Behçet’s disease in a child.J Korean Med Sci. 1987;2:129–132.
Yazici H, Yurdakul S, Hamuryudan V. Behçet’s syndrome.Curr Opin Rheumatol. 1999;11:53–57.
Slavin S, Nagler A, Varadi G, et al. Graft vs autoimmunity following allogeneic non-myeloablative blood stem cell transplantation in a patient with chronic myelogenous leukemia and severe systemic psoriasis and psoriatic polyarthritis.Exp Hematol. 2001;8:853–857.
Sakai H, Manabe A, Kojima S, et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan.Leukemia. 2001;15:1713–1720.
Locatelli F, Zecca M, Niemeyer C, et al. Roll of allogeneic bone marrow transplantation for the treatment of myelodysplastic syndromes in childhood.Bone Marrow Transpl. 1996;18(suppl 2): 63–68.
Gluckman E. Current status of umbilical cord blood hemato- poietic stem cell transplantation.Exp Hematol. 2000;28: 1197–1205.
Ohnuma K, Isoyama K, Ikuta K, et al. Cord blood transplantation from HLA-mismatched unrelated donors as a treatment for children with hematological malignancies.Br J Haematol. 2001;112: 981–987.
Tabata M, Tomomasa T, Kaneko H, et al. Intestinal Behçet’s disease: a case report and review of Japanese reports in children.J Pediatr Gastroenterol Nutr. 1999;29:477–481.
Anderson JE, Anasetti C, Frederick R, et al. Unrelated donor marrow transplantation for myelodysplasia (MDS) and MDS-related acute myeloid leukemia.Br J Haematol. 1996;93:59–67.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Yamato, K. Successful Cord Blood Stem Cell Transplantation for Myelodysplastic Syndrome with Behçet Disease. Int J Hematol 77, 82–85 (2003). https://doi.org/10.1007/BF02982607
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF02982607