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Hematological features of pediatric systemic lupus erythematosus: suggesting management strategies in children
Lupus, 06/08/2012
Gokce M et al. – Hematological findings should be carefully assessed and treated vigorously to prevent the morbidity and possible mortality.
Methods- The authors evaluated hematological findings of 43 children with SLE diagnosed and followed at the Pediatric Rheumatology Division of Hacettepe University, Turkey.
- Thirty–seven patients with hematological abnormalities were analyzed in detail.
- Median age at presentation was 13 years.
- Hematological involvement was seen in 86% of patients.
- The most common hematological finding was anemia (n=30).
- Anemia was either a Coombs (+) hemolytic one, or was due to other causes. Hemolytic anemia was treated with steroids and intravenous gamma globulin (IVIG).
- Leucopenia and thrombocytopenia were detected in 35.1 % and 37.8 %, respectively.
- Bone marrow aspiration was performed in 15, mainly for cytopenia.
- Secondary dysplastic changes were common.
- Acute lymphoblastic leukemia (ALL) was diagnosed in one patient.
- Six patients were diagnosed as having macrophage activation syndrome (MAS).
- One patient died due to secondary infections and multiorgan failure despite aggressive treatment. In patients diagnosed early, treatment with steroids and cyclosporine resulted in an excellent response.
- Thrombotic microangiopathy was detected in two patients.
- Both were treated successfully with steroids and plasma exchange.
- Antiphospholipid and anticardiolipin antibodies were positive in 12 and 15 of the patients, respectively.
- Five developed deep vein thrombosis (DVT), one cerebral sinus thrombosis and one presented with purpura fulminans.
- They were effectively treated with anticoagulation protocol.
