title: Bone marrow transplantation for Behçet's disease: a case report and systematic review of the literature.
abstract: OBJECTIVE
  Behçet's disease (BD) can be life threatening and may be refractory to corticosteroids and immunosuppressives. There has been some experience with haematopoietic stem cell transplantation (HSCT) in BD either for severe, refractory disease or for a haematological condition. The objectives of this study were to describe a BD patient undergoing HSCT and to evaluate the outcomes of BD patients who underwent HSCT.
  
  
  METHODS
  We report a BD patient with refractory gastrointestinal (GI) involvement who had HSCT for concomitant myelodysplastic syndrome (MDS). We also performed a systematic literature search regarding HSCT for either refractory disease or concomitant haematological conditions in BD patients.
  
  
  RESULTS
  A 30-year-old woman with refractory GI BD involvement with trisomy 8 MDS underwent a successful myeloablative allogeneic HSCT resulting in complete resolution of both BD and MDS. Additionally we identified 14 manuscripts providing data on 19 patients with BD who had HSCT. Among these 20 patients, including ours, refractory disease was the indication of transplantation in 9, while 11 patients were transplanted because of accompanying haematological conditions. Transplant indications for the nine patients (four male, five female) with refractory BD were neurological involvement in five, pulmonary artery aneurysm in two, GI disease in one and not reported in one patient. Three patients with neurological disease, both patients with pulmonary artery aneurysm and the patient with intestinal involvement achieved complete remission of their disease. Six patients transplanted for haematological conditions, including the presented case, also had GI involvement of BD. All of these patients achieved complete remission of GI findings after HSCT.
  
  
  CONCLUSIONS
  When considering HSCT, the potential adverse events and complications, which can be fatal, need to be kept in mind.
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Teoman
authors: T
authors: Soysal
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. aysesalihoglu@hotmail.com.
authors: Ayşe
authors: A
authors: Salihoğlu
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Sinem Nihal
authors: SN
authors: Esatoğlu
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Emine
authors: E
authors: Gültürk
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Ahmet Emre
authors: AE
authors: Eşkazan
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Gülen
authors: G
authors: Hatemi
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Ibrahim
authors: I
authors: Hatemi
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Şeniz
authors: Ş
authors: Öngören Aydın
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Yusuf Ziya
authors: YZ
authors: Erzin
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Zafer
authors: Z
authors: Başlar
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Nükhet
authors: N
authors: Tüzüner
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Burhan
authors: B
authors: Ferhanoğlu
authors: Division of Hematology, Division of Rheumatology, Division of Gastroenterology, Department of Internal Medicine and Division of Hematopathology, Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors: Aykut Ferhat
authors: AF
authors: Çelik
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000150:complications / Q000628:therapy*; D002898:Chromosomes, Human, Pair 8; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000235:genetics / Q000628:therapy; D016896:Treatment Outcome; D014314:Trisomy
keywords: Behçet’s disease; Behçet’s syndrome; blood stem cell transplantation; haematopoietic stem cell transplantation; myelodysplastic syndrome; refractory autoimmune disease