Obaji S, Alikhan R, Rayment R, Carter P, Macartney N, Collins P
Department of Haematology, School of Medicine, Cardiff University, Cardiff, UK.
Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
Haemophilia. 2016 Mar;22(2):285-291. doi: 10.1111/hae.12811. Epub 2015 Sep 30.
In a minority of patients with a significant bleeding history no cause is found despite extensive testing and we diagnose such cases as unclassified bleeding disorders (UBD). UBDs may have diverse underlying causes and currently no standard management strategy exists in the event of a haemorrhage or to cover surgery.
To document the clinical characteristics and response to treatment of UBDs.
We performed a retrospective chart review of all patients with UBDs who had an invasive procedure at our centre between 1998 and 2014.
The commonest symptoms were menorrhagia (89%) and bleeding at the time of surgery (88%) or dental extraction (85%). A total of 33 patients underwent 78 minor and major haemostatic challenges. Haemostatic cover was provided in 28 procedures with tranexamic acid alone, two with desmopressin and 45 with both agents in combination. A successful haemostatic outcome was observed in 70/78 (90%) cases. No patient required additional surgical intervention to achieve haemostasis, but one patient required a platelet transfusion to control postoperative bleeding.
This is the first study to report on the investigation and treatment of UBD. Future studies are needed to further our understanding of the bleeding phenotype and identify any underlying causes.
在少数有明显出血史的患者中，尽管进行了广泛检查仍未发现病因，我们将此类病例诊断为未分类出血性疾病（UBD）。UBD可能有多种潜在病因，目前在出血或手术时尚无标准的管理策略。
记录UBD的临床特征及治疗反应。
我们对1998年至2014年间在本中心接受侵入性操作的所有UBD患者进行了回顾性病历审查。
最常见的症状是月经过多（89%）以及手术时出血（88%）或拔牙时出血（85%）。共有33例患者接受了78次轻微和重大止血挑战。28例手术仅使用氨甲环酸进行止血覆盖，2例使用去氨加压素，45例两者联合使用。78例中有70例（90%）止血成功。没有患者需要额外的手术干预来实现止血，但有1例患者需要输注血小板来控制术后出血。
这是第一项报道UBD调查和治疗的研究。需要未来的研究来进一步了解出血表型并确定任何潜在病因。
