Arokszállási Ánita, Ilonczai Péter, Rázsó Katalin, Oláh Zsolt, Bereczky Zsuzsanna, Boda Zoltán, Schlammadinger Agota
Division of Thrombosis and Haemostasis, 2nd Department of Medicine, University of Debrecen, Hungary.
Blood Coagul Fibrinolysis. 2012 Oct;23(7):584-9. doi: 10.1097/MBC.0b013e3283551102.
Acquired haemophilia is a potentially life-threatening bleeding disorder. Its early diagnosis and treatment is of major importance. We evaluated the elapsed time between the first presentation of the bleeding symptoms and the correct diagnosis in the cases of the acquired haemophilia patients referred to our centre between 1999 and 2011. The causes and consequences of the often delayed diagnosis were also examined. The clinical and laboratory data of 13 patients with acquired haemophilia were analysed. Eleven patients had inhibitors to factor VIII (FVIII), in one case the autoantibody developed to factor XIII (FXIII) and in one to factor V (FV). The median period between the onset of the bleeding symptoms and the correct diagnosis was 1.5 months (3.0 days-9.0 months). In four cases 4.0-9.0 months were needed to establish the diagnosis. The main reason of this delay was that either the prothrombin time was used exclusively to evaluate haemostasis in primary care and also in some secondary care centres, or the prolonged activated partial thromboplastin time went unnoticed despite the obvious bleeding symptoms. Our observation underlines the importance of early referral of patients with unexplained bleeding symptoms to centres with appropriate laboratory facilities and experience in the diagnosis of bleeding disorders.
获得性血友病是一种潜在的危及生命的出血性疾病。其早期诊断和治疗至关重要。我们评估了1999年至2011年间转诊至我们中心的获得性血友病患者从首次出现出血症状到正确诊断所经过的时间。还研究了诊断经常延迟的原因及后果。分析了13例获得性血友病患者的临床和实验室数据。11例患者产生了针对凝血因子VIII(FVIII)的抑制物,1例患者的自身抗体针对凝血因子XIII(FXIII)产生,1例针对凝血因子V(FV)产生。从出血症状出现到正确诊断的中位时间为1.5个月(3.0天至9.0个月)。4例患者需要4.0至9.0个月才能确诊。这种延迟的主要原因是,在基层医疗以及一些二级医疗中心,仅使用凝血酶原时间来评估止血情况,或者尽管有明显的出血症状,但延长的活化部分凝血活酶时间却未被注意到。我们的观察强调了将有不明原因出血症状的患者尽早转诊至具备适当实验室设施且有出血性疾病诊断经验的中心的重要性。