Taddio Andrea, Brescia AnneMarie C, Lepore Loredana, Rose' Carlos D
Department of Sciences of Reproduction and Development, University of Trieste, IRCCS Burlo Garofolo, Istituto per l'Infanzia, via dell'Istria 65/1, Trieste, 34100, Italy.
Division of Rheumatology, A.I. duPont Hospital for Children, Department of Paediatrics, Thomas Jefferson University, 1600 Rockland Road, Wilmington, DE, 19803, USA.
Clin Rheumatol. 2007 Dec;26(12):2167-2169. doi: 10.1007/s10067-007-0647-5. Epub 2007 May 22.
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare acquired disorder associated with several different conditions but mostly with systemic lupus erythematosus (SLE). LAHPS probably results from the presence of anti-Factor II antibodies, which usually counterbalance the prothrombotic effect of the lupus anticoagulant (LAC). In fact, Factor II deficiency in SLE is invariably associated with the presence of LAC. No consensus exists for the treatment of LAHPS. Corticosteroids, with or without the addition of vitamin K or blood products, have been a successful first-line treatment. Immunoglobulin (IVIG) treatment has been shown to be effective in the setting of acute bleeding. However, in some patients, conservative treatment is not enough to control bleeding, and the addition of immunosuppressive therapy, usually azathioprine, is needed. In our patients, Factor II deficiency reappeared after tapering steroids. Both children achieved normal Factor II levels with cyclophosphamide. This effect was long-lasting, a phenomenon that has not been documented in children prior to this report.
狼疮抗凝物质低凝血酶原血症综合征（LAHPS）是一种罕见的获得性疾病，与多种不同病症相关，但大多与系统性红斑狼疮（SLE）有关。LAHPS可能是由抗凝血因子II抗体的存在所致，这些抗体通常会抵消狼疮抗凝物质（LAC）的促血栓形成作用。事实上，SLE中的凝血因子II缺乏总是与LAC的存在相关。对于LAHPS的治疗尚无共识。使用或不使用维生素K或血液制品的皮质类固醇一直是成功的一线治疗方法。免疫球蛋白（IVIG）治疗已被证明在急性出血情况下有效。然而，在一些患者中，保守治疗不足以控制出血，需要加用免疫抑制治疗，通常是硫唑嘌呤。在我们的患者中，减停类固醇后凝血因子II缺乏症再次出现。两名儿童使用环磷酰胺后凝血因子II水平恢复正常。这种效果是持久的，在此报告之前儿童中尚未有此现象的记录。
