Interhospital conference 8 กุมภาพันธ์ 2554 www.themegallery.com

History เด็กหญิง อายุ 7 ปี ภูมิลำเนา จ.หนองคาย CC: เลือดออกตามไรฟันและกำเดาไหล 2 เดือน PI: 2 เดือน เลือดกำเดาไหล เลือดออกตามไรฟัน ริมฝีปาก ออกนาน ออกทุกวัน มีไข้เป็นๆหายๆมีจุดจ้ำเลือดตามตัว เหนื่อยเพลียง่าย ซีดลง ประวัติครอบครัว: ปกติ www.themegallery.com

Physical examination Active, BW 30 kg, Ht 135 cm. BT 36.7oC, 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 www.themegallery.com

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Laboratory findings www.themegallery.com

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blood 3+, RBC >100/HPF, WBC 5-10/HPF, albumin 2+ Urine examination pH 5.5, sp. gr. 1.011, blood 3+, RBC >100/HPF, WBC 5-10/HPF, albumin 2+ www.themegallery.com

ANA - positive/fine speckle Lupus anticoagulant - positive C4 < 1.44 mg/dL C3 21 mg/dL Anti-DS DNA - positive Anti-SM - negative ANA - positive/fine speckle Lupus anticoagulant - positive www.themegallery.com

Patient Factor II 0.81% Factor V 67.27% Factor VII 140% Factor X 85.65% www.themegallery.com

Patient Factor II 0.81% Factor V 67.27% Factor VII 140% Factor X 85.65% Factor II Father 98% Mother 102.6% www.themegallery.com

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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. 2007 Galli M. Antiprothrombin antibodies: detection and clinical significance in the antiphospholipid syndrome. Blood. 1999 www.themegallery.com

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 www.themegallery.com

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. www.themegallery.com