论文部分内容阅读
目的对儿童获得性血友病A(AHA)的临床特点进行总结,以提高对该病的认识。方法对北京协和医院2002年9月和2008年12月收治的2例儿童AHA及医学引文索引(Medline)检索到的16例患儿,共18例,进行回顾性分析,并与成人获得性血友病的临床特点进行比较。结果儿童AHA发病年龄平均为6.6岁(2～14岁),从出现症状至确诊时间平均3.1个月(1周至2年)。7例(38.9%)可能与感染相关,如上呼吸道感染、咽炎、乳突炎、肺炎等;3例(16.6%)可能与青霉素使用相关;3例(16.6%)可能与肾脏疾病相关,如肾病综合征、Goodpasture综合征等;2例(11.1%)可能与自身免疫性疾病相关,包括系统性红斑狼疮、幼年型类风湿性关节炎;3例(16.6%)无相关基础疾病。临床表现主要为皮肤大片瘀斑(61.1%,11/18)和肌肉或软组织血肿(55.6%,10/18),关节出血相对少见,占11.1%(2/18)。7例(38.9%)患儿经过原发病治疗、替代治疗和(或)免疫抑制治疗,在1～6周内获得缓解(CR),8例(44.4%)在3～60个月获得CR,总CR率达83.3%,2例(11.1%)患儿死亡。活化的部分凝血活酶时间(APTT)、凝血因子Ⅷ活性(FⅧ∶C)及凝血因子Ⅷ抑制物(FⅧ∶Ab)滴度水平与临床出血严重程度以及获得CR的时间均无明确相关性(P>0.05)。结论儿童AHA极罕见,常存在基础疾病,皮肤和软组织出血为其主要临床表现。掌握其临床特点及诊断流程有助于早期诊断、减少误诊。在积极治疗基础疾病的同时,给与替代治疗,必要时加用糖皮质激素或免疫抑制剂,能使本病获得长期CR或治愈。
Objective To summarize the clinical features of children-acquired haemophilia A (AHA) in order to improve their understanding of the disease. Methods A total of 16 children were retrieved from two children with AHA and Medline from September 2003 to December 2008 in Peking Union Medical College Hospital. A total of 18 children were retrospectively analyzed and compared with adults with acquired blood Friends of the clinical features of the disease were compared. Results The mean age at onset of AHA in children was 6.6 years (range, 2-14 years). The mean onset time to symptom onset was 3.1 months (1 week to 2 years). Seven cases (38.9%) were related to infection such as upper respiratory tract infection, pharyngitis, mastoiditis and pneumonia. Three cases (16.6%) were related to penicillin use and three cases (16.6%) were related to renal diseases such as nephropathy Syndrome, Goodpasture syndrome, etc .; 2 cases (11.1%) may be related to autoimmune diseases, including systemic lupus erythematosus, juvenile rheumatoid arthritis; 3 cases (16.6%) without underlying diseases. The major clinical manifestations were ecchymosis of the skin (61.1%, 11/18) and muscle or soft tissue hematoma (55.6%, 10/18). Joint bleeding was relatively uncommon, accounting for 11.1% (2/18). Seven patients (38.9%) had remission (CR) within 1 ~ 6 weeks and 8 (44.4%) patients with CR after 3 ~ 60 months after treatment of primary disease, replacement therapy and / or immunosuppressive therapy , The total CR rate reached 83.3%, 2 cases (11.1%) of children died. The levels of activated partial thromboplastin time (APTT), factor Ⅷ activity (F Ⅷ: C), and factor Ⅷ inhibitor (F Ⅷ: Ab) were not significantly correlated with the severity of clinical bleeding and the time to CR P> 0.05). Conclusion The AHA in children is extremely rare and often presents with underlying diseases. Skin and soft tissue hemorrhage are the main clinical manifestations. Grasp its clinical features and diagnostic processes help early diagnosis and reduce misdiagnosis. In the active treatment of underlying diseases at the same time, to give alternative treatment, if necessary, add glucocorticoid or immunosuppressive agents, can make the disease to obtain long-term CR or cure.