Sarker T, Roy S, Hollon W, Rajpurkar M
Division of Hematology/Oncology, Carman and Ann Adams, Department of Pediatrics, Children's Hospital of Michigan, Detroit, MI, USA.
Wayne State University, Detroit, MI, USA.
Haemophilia. 2015 Nov;21(6):754-60. doi: 10.1111/hae.12669. Epub 2015 Apr 30.
Lupus anticoagulant associated with acquired prothrombin deficiency also known as 'lupus anticoagulant hypoprothrombinemia syndrome' (LAHS) is an entity that is well described in adults and is usually associated with autoimmune conditions (LAHS-AI). However, in children, LAHS has unique features that are distinct from the adult type.
We report two paediatric cases of LAHS, describe their distinct patterns and review the paediatric literature on LAHS.
Case studies on two patients with LAHS were reviewed, details on their presentation, work up and management were extracted. A Medline search was conducted on LAHS in children between 1960 and 2014; Articles in languages other than English were excluded.
The case studies highlight the differences in the two patterns of childhood LAHS. Additionally the review of the literature reveals that there are 15 case reports and 5 case series that report 25 children with LAHS-AI, 9 case reports and 6 case series report 26 children of LAHS associated with viral infections (LAHS-VI). At presentation, all patients with LAHS-AI had positive laboratory tests for autoimmune diseases, most commonly for systemic lupus erythematosus while these tests were negative in LAHS-VI. All patients with LAHS-AI had a protracted course and needed prolonged treatment with immune-suppressive therapy while patients with LAHS-VI resolved spontaneously or needed short-term immune-modulating therapy.
In childhood, two distinct patterns of LAHS are observed, either associated with infection or autoimmune disease. Initial diagnostic investigations are critical to differentiating these two patterns as the prognosis and outcome for each is distinct.
狼疮抗凝物与获得性凝血酶原缺乏相关，也被称为“狼疮抗凝物低凝血酶原血症综合征”（LAHS），这一实体在成人中已有充分描述，通常与自身免疫性疾病相关（LAHS-AI）。然而，在儿童中，LAHS具有与成人类型不同的独特特征。
我们报告两例儿童LAHS病例，描述其独特模式并回顾关于LAHS的儿科文献。
回顾了两例LAHS患者的病例研究，提取了他们的临床表现、检查和治疗细节。对1960年至2014年间儿童LAHS进行了医学文献检索；排除了非英文文献。
病例研究突出了儿童LAHS两种模式的差异。此外，文献回顾显示有15篇病例报告和5个病例系列报道了25例患有LAHS-AI的儿童，9篇病例报告和6个病例系列报道了26例与病毒感染相关的LAHS儿童（LAHS-VI）。在就诊时，所有LAHS-AI患者的自身免疫性疾病实验室检查均为阳性，最常见的是系统性红斑狼疮，而这些检查在LAHS-VI中为阴性。所有LAHS-AI患者病程迁延，需要长期免疫抑制治疗，而LAHS-VI患者可自发缓解或需要短期免疫调节治疗。
在儿童期，观察到LAHS有两种不同模式，分别与感染或自身免疫性疾病相关。初始诊断性检查对于区分这两种模式至关重要，因为每种模式的预后和结局都不同。
