This is a case of lupus anticoagulant hypoprothrombinemia syndrome associated with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus

초록

A 17-year-old girl with SLE presented with squeezing colicky abdominal pain for a day (2018.12). She had been diagnosed with SLE, based on ecchymosis and prolonged bleeding after tooth extraction at 9 years of age (2011.9). About one year later she developed proteinuria (10.7 mg/m2/hr) and renal biopsy showed focal segmental proliferative lupus glomerulonephritis Class IIIA. At that time, the patient had positive results for the lupus anticoagulant (LA) and immunoglobulin G (IgG)/IgM anti-phospholipid antibodies with prolonged activated partial thromboplastin time (aPTT) and normal international normalized ratio (INR) (2012.10). She had taken a combination of mycophenolate mofetil (MMF), prednisolone, azathioprine, enalapril or hydroxychloroquine. On physical examination, her vital signs were within normal limits, and epigastric and generalized abdominal tenderness was found without hepatosplenomegaly. Coagulation studies revealed INR and aPTT prolongation and LA positivity. Levels of C3 and C4 were decreased. She had positive fndings for anti-cardiolipin antibodies (aCL) IgG and anti-beta-2-glycoprotein I (β2GP1) IgG/IgM. Due to abdominal discomfort and suspected SLE faring, MMF dose was reduced and steroid dose was increased. Three months later (2019.3), she complained of dizziness and menorrhagia. Red blood cell transfusion was performed at a hemoglobin level of 7.4 g/dL. Laboratory tests showed prolonged aPTT and INR, LA positivity, and positive fndings for aCL IgG and anti-β2GP1 IgG/IgM again. Proteinuria was aggravated from 519 mg/day to 1,204 mg/day. Pelvic sonography showed decreasing sized ovarian cystic lesion (< 2 cm). For the control of menorrhagia, combined oral contraceptive pill was given for 3 months. Repeated coagulation studies showed persistently prolonged aPTT (the longest 106.8 sec) and low factor II level (the lowest 14%). A diagnosis of LAHPS associated with SLE was made. She received six courses of cyclophosphamide pulse therapy (monthly 6 times). After that, she is currently taking cyclosporine, prednisolone, hydroxychloroquine, and enalapril.