Successful engraftment of unrelated cord blood stem cells for familial erythrophagocytic lymphohistiocytosis. Kinki Cord Blood Bank

PURPOSE: To describe a case of Felty syndrome (FS) in a child with (JRA) and review the previous literature on this rare entity. METHODS: Review of clinical data including results of serial blood counts, bone marrow aspirate, human leukocyte antigen (HLA)-typing, and abdominal sonography. RESULTS: Serial blood counts over 2 years revealed persistent leukopenia and thrombocytopenia. Bone marrow aspirate showed normal trilineage hematopoiesis, abdominal sonography demonstrated an enlarged spleen, but normal liver and portal circulation. HLA-typing was most significant for positivity of the DR 1 allele. CONCLUSION: This is only the third child, and the first preadolescent, to be reported with FS complicating juvenile rheumatoid arthritis. This condition needs to be considered in the differential diagnosis when leukopenia, thrombocytopenia, or both develop in patients with JRA.