Physical examination Active, BW 30 kg, Ht 135 cm. BT 36.7 o C, PR 120/min, RR 20/min, BP 120/73 mmHg mildly pale, not icteric, CLN - impalpable Abdomen : soft, no hepatosplenomegaly Ext : old ecchymoses, no petechiae
Urine examination pH 5.5, sp. gr , blood 3+, RBC >100/HPF, WBC 5-10/HPF, albumin 2+
C 4 < 1.44 mg/dL C 3 21 mg/dL Anti-DS DNA - positive Anti-SM - negative ANA - positive/fine speckle Lupus anticoagulant - positive
Patient Factor II 0.81% Factor V 67.27% Factor VII 140% Factor X 85.65%
Patient Factor II 0.81% Factor V 67.27% Factor VII 140% Factor X 85.65% Factor II Father 98% Mother 102.6%
Reports Erkan D. Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus: report of 2 cases and review of literature. Lupus 1999 Taddio A. Steady improvement of prothrombin levels after cyclophosphamide therapy in pediatric lupus anticoagulant hypoprothrombinemia syndrome (LAHPS). Clin Rheumatol Galli M. Antiprothrombin antibodies: detection and clinical significance in the antiphospholipid syndrome. Blood
Lupus anticoagulant-hypoprothrombinemia syndrome Bleeding - rare manifestation of antiphospholipid syndrome nearly always due to factor II deficiency Mechanism: non-neutralizing antibodies bind prothrombin, resulting in the rapid clearance of prothrombin-anti-prothrombin antibody complexes from circulation
Lupus anticoagulant-hypoprothrombinemia syndrome SLE/healthy children after viral infection Most of patients either had LA-HPS at initial diagnosis of SLE or within a year of onset of symptoms Corticosteroid are to decrease the clearance of the prothrombin-antiprothrombin antibody complexes Most of the cases responded successfully to steroids.