title: A reappraisal of the association between Behçet's disease, myelodysplastic syndrome and the presence of trisomy 8: a systematic literature review.
abstract: OBJECTIVE
  A number of patients with Behçet's disease (BD) associated with myelodysplastic syndrome (MDS) with or without trisomy 8 have been reported. A high frequency of gastrointestinal (GI) involvement was reported in such patients. The aim of this systematic literature review was to delineate whether GI involvement is an inherent feature of BD associated with MDS, whether these patients do actually have BD rather than GI symptoms related to MDS, and whether the presence of trisomy 8 plays a role in the disease expression of BD associated with MDS.
  
  
  METHODS
  A systematic literature review was performed in PubMed using the keywords (Behçet's disease OR Behçet's syndrome) AND (myelodysplastic syndrome OR trisomy 8) until December 2013.
  
  
  RESULTS
  Data from 39 manuscripts that met the inclusion criteria, reporting on 52 patients were analysed. GI involvement was common in reports from both the Far East and non-Far East countries (75% vs. 50.0%, p=0.15). These patients had typical BD manifestations, except for 1 patient who had only oral ulcers and gastrointestinal involvement. The presence of trisomy 8 seems to be associated with an increased frequency of fever (79.5% vs. 33.3%, p=0.005).
  
  
  CONCLUSIONS
  GI involvement seems to be an inherent feature of BD associated with MDS regardless of geographic differences. Despite the increased frequency of GI involvement in these patients, MDS does not seem to modify the clinical expression of gastrointestinal involvement. Presence of trisomy 8 seems to modify the disease expression with an increased frequency of fever.
authors: Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
authors: Sinem Nihal
authors: SN
authors: Esatoglu
authors: Division of Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. gulenhatemi@yahoo.com.
authors: Gulen
authors: G
authors: Hatemi
authors: Division of Haematology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
authors: Ayse
authors: A
authors: Salihoglu
authors: Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
authors: Ibrahim
authors: I
authors: Hatemi
authors: Division of Haematology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
authors: Teoman
authors: T
authors: Soysal
authors: Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
authors: Aykut Ferhat
authors: AF
authors: Celik
mesh_terms: D001528:Behcet Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D005767:Gastrointestinal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D006801:Humans; D009190:Myelodysplastic Syndromes / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D010641:Phenotype; D011379:Prognosis; D012307:Risk Factors; D014314:Trisomy / Q000235:genetics*