Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
复制标题
狼疮抗凝低凝血酶原血症综合征和免疫球蛋白 A 血管炎：日本兄弟姐妹病例报告及文献综述。
DOI：
10.1007/s00296-019-04404-7
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Shimada A.
中科院分区：
文献类型：
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作者：
Fujiwara K;Shimizu J;Tsukahara H;Shimada A.
狼疮抗凝血-低凝血酶原血症综合征（LAHPS）是一种罕见的出血性疾病引起的抗凝血酶原抗体。LAHPS与系统性红斑狼疮（SLE）或感染有关。我们描述了两个日本兄弟与免疫球蛋白-A血管炎（IgAV）谁符合诊断标准的LAHPS。他们表现为可触及的紫癜和腹痛，并有延长的活化部分凝血活酶时间（APTT）和凝血酶原缺乏症与狼疮抗凝剂的存在。在1996年至2019年期间，在日本医学文摘协会的摘要中对儿科LAHPS进行了综述，这些摘要以日语撰写，并以英文撰写PubMed或Web of Science列出的文章。包括我们的病例，在40例日本和46例非日本患者中报告了儿科LAHPS。我们总结了所有86例病例的临床和实验室特征，发现除我们的病例外，只有一例日本LAHPS伴IgAV。在86例病例中，大多数与感染有关，其次是SLE。存在SLE、年龄较大、凝血酶原水平较低、严重出血症状、免疫球蛋白G抗心磷脂抗体和抗心磷脂/β2-糖蛋白I抗体和/或β2-糖蛋白I依赖性抗心磷脂抗体阳性的患者需要治疗的几率较高。当IgAV患者没有典型的临床过程或独特的症状时，可能需要测量APTT和凝血酶原时间（PT）。LAHPS应考虑APTT和/或PT延长。此外，在接受治疗的LAHPS病例中，当PT和FIB水平正常化时，维持血栓形成和出血风险之间的平衡非常重要。
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare bleeding disorder caused by antiprothrombin antibodies. LAHPS is associated with systemic lupus erythematosus (SLE) or infections. We describe two Japanese brothers with immunoglobulin-A vasculitis (IgAV) who met the diagnostic criteria of LAHPS. They presented with palpable purpura and abdominal pain, and had a prolonged activated partial thromboplastin time (APTT) and prothrombin deficiency with the presence of lupus anticoagulant. Pediatric LAHPS was reviewed in abstracts from the Japan Medical Abstracts Society that were written in Japanese and PubMed or Web of Science-listed articles in English between 1996 and 2019. Including our cases, pediatric LAHPS has been reported in 40 Japanese and 46 non-Japanese patients. We summarized the clinical and laboratory characteristics of all 86 cases, and found only one Japanese LAHPS case with IgAV, except for our cases. Of the 86 cases, most were associated with infections followed by SLE. The presence of SLE, older age, lower prothrombin levels, severe bleeding symptoms, and positivity of immunoglobulin G anticardiolipin antibodies and anticardiolipin/β2-glycoprotein I antibodies and/or β2-glycoprotein I-dependent anticardiolipin antibodies had higher odds of requiring treatment. Measuring the APTT and prothrombin time (PT) might be required in patients with IgAV when they do not have a typical clinical course or distinctive symptoms. LAHPS should be considered with prolongation of the APTT and/or PT. Additionally, it is important to maintain a balance between the risk of thrombosis and hemorrhage when normalization of the PT and FII levels occurs in LAHPS cases under treatment.