Division of Hematology and Oncology, Department of Internal Medicine, Vanderbilt University Medical Center, Nashville, TN 37232-6307, USA.
Haemophilia. 2013 Mar;19(2):e62-5. doi: 10.1111/hae.12027. Epub 2012 Sep 19.
Hypoprothrombinemia associated with a lupus anticoagulant (LA) was first reported in the literature over 50 years ago. The hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare bleeding diathesis that has been associated with LAs in adult and paediatric patients with systemic lupus erythematosus (SLE) and with transient LAs due to other causes. There are no standard recommendations for treating haemorrhage associated with this syndrome. Herein, we report a patient with SLE and HLAS who achieved a durable remission following treatment with intravenous immune globulin (IVIG), prednisone and rituximab.
50 多年前，文献中首次报道了与狼疮抗凝物（LA）相关的低凝血酶原血症。低凝血酶原血症-狼疮抗凝物综合征（HLAS）是一种罕见的出血倾向，与系统性红斑狼疮（SLE）成年和儿童患者的 LA 以及由于其他原因导致的一过性 LA 相关。目前，对于治疗与该综合征相关的出血，尚无标准的推荐治疗方案。在此，我们报告了一例 SLE 合并 HLAS 患者，经静脉注射免疫球蛋白（IVIG）、泼尼松和利妥昔单抗治疗后，达到了持久缓解。
