title: Hematological features of pediatric systemic lupus erythematosus: suggesting management strategies in children.
abstract: OBJECTIVE
  The aim of this study was to analyze the hematological features in children with systemic lupus erythematosus (SLE) and to review our current treatment protocols.
  
  
  METHODS
  We 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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Hematological findings should be carefully assessed and treated vigorously to prevent the morbidity and possible mortality.
authors.affiliation: Hacettepe University, School of Medicine, Department of Pediatric Hematology, Ankara, Turkey.
authors.forename: M
authors.initials: M
authors.lastname: Gokce
authors.forename: Y
authors.initials: Y
authors.lastname: Bilginer
authors.forename: N
authors.initials: N
authors.lastname: Besbas
authors.forename: F
authors.initials: F
authors.lastname: Ozaltin
authors.forename: M
authors.initials: M
authors.lastname: Cetin
authors.forename: F
authors.initials: F
authors.lastname: Gumruk
authors.forename: S
authors.initials: S
authors.lastname: Ozen
mesh_terms: D000293:Adolescent; D000743:Anemia, Hemolytic / Q000188:drug therapy / Q000209:etiology; D000906:Antibodies / Q000097:blood; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000188:drug therapy / Q000209:etiology; D002308:Cardiolipins / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007970:Leukopenia / Q000188:drug therapy / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000150:complications / Q000188:drug therapy*; D055501:Macrophage Activation Syndrome / Q000209:etiology / Q000628:therapy; D008297:Male; D010743:Phospholipids / Q000276:immunology; D010951:Plasma Exchange; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000209:etiology; D013256:Steroids / Q000627:therapeutic use; D057049:Thrombotic Microangiopathies / Q000209:etiology / Q000628:therapy; D020246:Venous Thrombosis / Q000188:drug therapy / Q000209:etiology
chemical_list: D000906:Antibodies; D000925:Anticoagulants; D002308:Cardiolipins; D006495:Heparin, Low-Molecular-Weight; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D010743:Phospholipids; D013256:Steroids; D016572:Cyclosporine