Department of Internal Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Mod Rheumatol Case Rep. 2021 Jul;5(2):306-309. doi: 10.1080/24725625.2021.1920139. Epub 2021 May 10.
We describe the case of a 50-year-old woman with a history of SLE and APS that presented with a spontaneous subdural haematoma, prolonged aPTT, PT and INR and positive LA. The activity of the coagulation factors II, VIII, IX and XI was extremely low, and anti-prothrombin antibody IgG was positive. LAHS was established, with inhibition of the intrinsic pathway, as an acquired haemophilia. The patient received corticosteroids and cyclophosphamide as treatment. To the best of our knowledge, this is one of the few reports of spontaneous intracranial bleeding, an unusual and initial manifestation of LAHS in an adult patient.HighlightsLAHS is characterised by the presence of LA and hypoprothrombinaemia caused by anti-prothrombin antibodies.Prolonged aPTT and INR, and positive LA are important laboratory findings that help the suspicion of LAHS.Intracranial bleeding is an unusual manifestation of LAHS associated with low factor II activity.Corticosteroids are the first-line treatment of LAHS.The prognosis of LAHS is good with adequate treatment, with a reported mortality of 5%.
我们描述了一例 50 岁女性，有 SLE 和 APS 病史，表现为自发性硬脑膜下血肿、延长 aPTT、PT 和 INR 以及 LA 阳性。凝血因子 II、VIII、IX 和 XI 的活性极低，抗凝血酶原抗体 IgG 阳性。诊断为获得性血友病伴抑制内源性途径的狼疮抗凝物相关性脑病（LAHS）。患者接受了皮质类固醇和环磷酰胺治疗。据我们所知，这是少数几例自发性颅内出血报告之一，也是成人 LAHS 中一种不常见和初始表现。
LAHS 的特征是存在狼疮抗凝物和抗凝血酶原抗体引起的低凝血酶原血症。延长的 aPTT 和 INR 以及阳性的 LA 是有助于怀疑 LAHS 的重要实验室发现。颅内出血是与低因子 II 活性相关的 LAHS 的不常见表现。皮质类固醇是 LAHS 的一线治疗药物。在充分治疗的情况下，LAHS 的预后良好，死亡率为 5%。
