Abstract
This research aimed to study the frequency and the long-term outcome of initial hematological abnormalities in pediatric-Systemic Lupus Erythematosus (p-SLE) among Saudi children. Thirty out of 36 children with SLE (diagnosed according to the revised American College of Rheumatology criteria, between 2000-2011) with hematological abnormalities were included in this prospective study conducted at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. A frequency of 83% (30/36 patients) was estimated for hematological abnormalities in p-SLE. This frequency is much higher than that found in several other studies. Leukopenia occurred in 40% of the children at presentation and in 13.3% during the course of the illness. Eighty percent of patients (29/36) presented with a normochromic normocytic anemia. Fifteen patients had iron deficiency with a hypochromic microcytic anemia following disease remission at follow up. Other hematologic abnormalities included a Coombs positive hemolytic anemia at presentation in 27%, thrombocytopenia in 20%, and antiphospholipid antibodies in 9%. Most of these hematological abnormalities improved significantly with systemic steroid and immunosuppressive agents (azathioprine, cyclophosphamide, and mycofenolate). One patient with a positive anticardiolipin antibody and lupus anticoagulant experienced thrombotic events. Hematological abnormalities were common in p-SLE, but the course was generally benign. Recurrent hemolysis and steroid toxicity in patients with AIHA remain a challenge for rheumatologists. An evaluation for SLE is critical in children presenting with unexplained hematological abnormalities.