Juvenile idiopathic arthritis with myelofibrosis

Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy of children. JIA comprises a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and has symptoms lasting for at least 6 weeks. Childhood myelofibrosis (MF) is rare with variable outcome reported in literature. MF with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoiesis, and varying degrees of MF. It may be idiopathic or secondary to a large number of conditions. Autoimmune MF has been commonly reported in association with systemic lupus erythematosus. MF presenting as cytopenias has been reported in immune mediated disorders such as rheumatoid arthritis, ulcerative colitis, primary biliary cirrhosis, polyarteritis nodosa, and Sjogren's syndrome. A case of MF in a 14 year old boy who presented with anemia, pancytopenia, splenomegaly, and oligo articular arthritis is described which as per the literature falls into the rarest of cases to be found. This case is only the second reported case of MF associated with JIA in world literature, the only previous case being reported by Jain et al . in 2005 from India.