Lupus anticoagulant and transient severe bleeding tendency in a girl aged nine years.
A 9-year old girl admitted in our clinic for severe hemorrhagic syndrome was found to display a high level of lupus anticoagulant (LA) leading to an important prolongation of phospholipid-dependent coagulation. Positive antinuclear and anti DNA antibodies, as well as very low levels of complement C3 and C4 proteins confirmed the diagnosis of systemic lupus erythematosus. Therapy with cortisone and cyclophosphamide led to normalization of the clotting tests but could not arrest the development of renal and hepatic lesions. The patient is one of the few cases with presence of lupus anticoagulant associated with severe hemorrhagic diathesis, in opposition to the more frequently reported thrombotic tendency connected with antiphospholipid antibodies.
D000208:Acute Disease; D001323:Autoantibodies / Q000032:analysis*; D001379:Azathioprine / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000032:analysis / Q000276:immunology*; D002648:Child; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006474:Hemorrhagic Disorders / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D006854:Hydrocortisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D016682:Lupus Coagulation Inhibitor; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy; D010743:Phospholipids / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use
D001323:Autoantibodies; D001779:Blood Coagulation Factors; D003176:Complement C3; D003181:Complement C4; D016682:Lupus Coagulation Inhibitor; D010743:Phospholipids; D003520:Cyclophosphamide; C007133:hydrocortisone hemisuccinate; D001379:Azathioprine; D011241:Prednisone; D006854:Hydrocortisone