Myelodysplastic syndrome with trisomy 8 associated with Behçet syndrome: an immunologic link to a karyotypic abnormality.
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.
Thachil
Jecko V
JV
Department of Haematology, Royal Liverpool University Hospital, Liverpool, United Kingdom. jeckothachil@yahoo.co.uk
Salim
Rahuman
R
Field
Anne
A
Moots
Robert
R
Bolton-Maggs
Paula
P
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
D007166:Immunosuppressive Agents; D013792:Thalidomide; D016559:Tacrolimus