Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, 1757-Kanazawa, Ishikari, Tobetsu, Hokkaido, 061-0293, Japan.
Division of Clinical Laboratory, Health Sciences University Hospital, Tobetsu, Japan.
Int J Hematol. 2019 Aug;110(2):197-204. doi: 10.1007/s12185-019-02674-2. Epub 2019 Jun 4.
Patients with lupus anticoagulant (LA), a thrombotic risk factor, along with decreased prothrombin (FII) activity are classified as lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) and occasionally show bleeding symptoms, although this is not essential for diagnosis. We treated 20 cases of LAHPS over a 3-year period. Median FII activity was 20.9% and the anti-prothrombin antibody (anti-II Ab), shown by ELISA findings, was detected in 55%. Bleeding symptoms were observed in 20%, although that finding was not correlated with FII activity or anti-FII Ab quantity. We also observed 21 LA cases with decreased activity of coagulation factors other than FII, which we have designated LAHPS-like syndrome (LLS). Among LLS patients, anti-FII Ab and bleeding symptoms were seen in 47.6% and 14.3%, respectively. Our findings suggest that bleeding in LAHPS and LLS cannot be explained only by FII activity decreased by anti-FII Ab. Low FVIII activity and the anti-FVIII antibody (anti-FVIII Ab) were detected in some LAHPS and LLS patients, making it difficult to distinguish those from acquired hemophilia A cases. Detection of anti-FVIII Ab quantity by ELISA may be useful for accurate determination, as that was not performed in our LAHPS or LLS patients.
患有狼疮抗凝物 (LA) 的患者存在血栓形成风险因素，同时伴有凝血酶原 (FII) 活性降低，此类患者被归类为狼疮抗凝物-低凝血酶原血症综合征 (LAHPS)，偶尔会出现出血症状，但这并非诊断所必需。我们在 3 年的时间里治疗了 20 例 LAHPS 患者。FII 活性的中位数为 20.9%，通过 ELISA 检测发现，有 55%的患者存在抗凝血酶原抗体 (抗-II Ab)。虽然出血症状与 FII 活性或抗-FII Ab 量无相关性，但仍有 20%的患者出现了出血症状。我们还观察到 21 例除 FII 外其他凝血因子活性降低的 LA 病例，我们将其命名为 LAHPS 样综合征 (LLS)。在 LLS 患者中，分别有 47.6%和 14.3%的患者出现了抗-FII Ab 和出血症状。我们的研究结果表明，LAHPS 和 LLS 中的出血不能仅用抗-FII Ab 导致的 FII 活性降低来解释。一些 LAHPS 和 LLS 患者存在低 FVIII 活性和抗 FVIII 抗体 (抗-FVIII Ab)，这使得这些患者与获得性血友病 A 患者难以区分。通过 ELISA 检测抗-FVIII Ab 量可能有助于准确判断，因为我们的 LAHPS 或 LLS 患者并未进行此项检测。
