Favier Rémi, Kheyar Tassadit, Renolleau Sylvain, Tabone Marie Dominique, Favier Marie, Ulinski Tim
Hôpitaux de Paris, Service d'hématologie biologique, Hôpital d'enfants Armand Trousseau, Paris.
Ann Biol Clin (Paris). 2012 Mar-Apr;70(2):226-30. doi: 10.1684/abc.2012.0690.
We report a case of lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) in an 11 year old girl initially hospitalized for bleeding. The patient presented with petechia, persisting bleeding after tooth extraction performed two days before, nephritic syndrome (renal failure, proteinuria and macroscopic hematuria), severe anemia, thrombocytopenia, lymphopenia. The association of these abnormalities suggested LAHPS secondary to severe systemic lupus. Immediate treatment with fresh frozen plasma and intravenous immunoglobulins (400 mg/kg/5d) was started and followed by steroid (500 mg/d) and cyclophosphamide (800 mg/m(2)) pulse therapy leading to rapid improvement of bleeding, renal involvement and prothrombin levels within 13 days. Lupus diagnosis was confirmed by immunological investigations and renal biopsy. Two early relapses occurred despite adequate treatment. After a follow-up of two years, no further disease activity is noted while the patient is treated only by mycophenolate mofetil (1 200 mg/m(2)/d). LAHPS did not relapse during this follow-up.
我们报告了一例狼疮抗凝物-低凝血酶原血症综合征（LAHPS），患者为一名11岁女孩，最初因出血住院。患者出现瘀点，在两天前拔牙后持续出血，患有肾病综合征（肾衰竭、蛋白尿和肉眼血尿）、严重贫血、血小板减少、淋巴细胞减少。这些异常情况提示LAHPS继发于严重的系统性红斑狼疮。立即开始用新鲜冰冻血浆和静脉注射免疫球蛋白（400mg/kg/5天）进行治疗，随后进行类固醇（500mg/d）和环磷酰胺（800mg/m²）脉冲治疗，在13天内出血、肾脏受累及凝血酶原水平迅速改善。通过免疫学检查和肾活检确诊为狼疮。尽管治疗充分，仍发生了两次早期复发。经过两年的随访，在仅用霉酚酸酯（1200mg/m²/d）治疗期间未观察到进一步的疾病活动。在此随访期间LAHPS未复发。
