title: Myelodysplastic syndrome with trisomy 8 associated with Behçet syndrome: an immunologic link to a karyotypic abnormality.
abstract: Myelodysplastic syndrome (MDS) in children is often associated with chromosomal anomalies and trisomy 8 is a characteristic karyotypic feature in up to 20% of the cases. Behçet disease is a rare multisystem inflammatory disorder characterized by recurrent mouth and genital ulcers. MDS with trisomy 8 has been observed in adult patients with Behçet syndrome with some cases developing prior to the clinical manifestations of the latter. We present a female with a similar association and explain the importance of identifying the coexisting conditions. The immunological abnormalities, which may be observed in MDS and their possible mechanisms, are also discussed.
authors.affiliation: Department of Haematology, Royal Liverpool University Hospital, Liverpool, United Kingdom. jeckothachil@yahoo.co.uk
authors.forename: Jecko V
authors.initials: JV
authors.lastname: Thachil
authors.forename: Rahuman
authors.initials: R
authors.lastname: Salim
authors.forename: Anne
authors.initials: A
authors.lastname: Field
authors.forename: Robert
authors.initials: R
authors.lastname: Moots
authors.forename: Paula
authors.initials: P
authors.lastname: Bolton-Maggs
mesh_terms: D000293:Adolescent; D000753:Anemia, Refractory / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D002898:Chromosomes, Human, Pair 8; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007621:Karyotyping; D019226:Oral Ulcer / Q000188:drug therapy / Q000209:etiology; D016559:Tacrolimus / Q000627:therapeutic use; D013792:Thalidomide / Q000627:therapeutic use; D014314:Trisomy
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide; D016559:Tacrolimus