Successful cord blood stem cell transplantation for myelodysplastic syndrome with Behçet disease.

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.