Hematological abnormalities at presentation in pediatric systemic lupus erythematosus in Saudi children: Long-term outcome

Article type: Research Article

Authors: Muzaffer, Mohammed A.

Affiliations: Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, PO Box 80215, Jeddah 215 89, Saudi Arabia. Tel.: +966 2 640 83 27; Fax: +966 2 6408353; E-mail: mmuzaffer@hotmail.com

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.

Keywords: Pediatric Systemic Lupus Erythematosus (p-SLE), Autoimmune Hemolytic Anemia (AIHA)

DOI: 10.3233/JPB-2012-00053

Journal: Journal of Pediatric Biochemistry, vol. 2, no. 3, pp. 153-157, 2012