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成人出血性疾病的识别与基本管理

Identification and basic management of bleeding disorders in adults.

作者信息

Kruse-Jarres Rebecca, Singleton Tammuella C, Leissinger Cindy A

机构信息

From the Department of Medicine (RKJ, CAL), and the Department of Pediatrics (TCS), Tulane University School of Medicine, New Orleans, Louisiana.

出版信息

J Am Board Fam Med. 2014 Jul-Aug;27(4):549-64. doi: 10.3122/jabfm.2014.04.130227.

Abstract

Adults with bleeding disorders may present to their family physician with minor bleeding symptoms or hematologic laboratory abnormalities discovered during evaluation for surgery or another purpose. Identifying the small proportion of adults who have an underlying bleeding disorder as the cause for such signs or symptoms may be challenging. In cases of asymptomatic hematologic laboratory abnormalities, the particular abnormality should narrow down the potentially affected hemostatic component(s), ideally streamlining subsequent investigation. In patients presenting with bleeding symptoms, a thorough history and physical examination are critical for first identifying bleeding as pathologic, then performing the appropriate diagnostic evaluation after excluding identifiable causes. Knowledge of the pathophysiologic processes contributing to impaired hemostasis in any given bleeding disorder ensures proper treatment and avoids therapies that are unnecessary or even contraindicated. Management is further determined by bleeding phenotype and, for invasive procedures, the anticipated risk for bleeding. Consultation with a hematologist may facilitate proper evaluation and treatment, particularly in adults with rare bleeding disorders or no identifiable cause for bleeding. This article reviews the diagnostic approach to hematologic laboratory abnormalities and abnormal bleeding in adults, as well as basic preventive care and hemostatic management of adults with bleeding disorders.

摘要

患有出血性疾病的成年人可能因轻微出血症状或在手术评估或其他检查中发现的血液学实验室异常情况而就诊于家庭医生。要确定少数患有潜在出血性疾病的成年人是这些体征或症状的病因可能具有挑战性。对于无症状的血液学实验室异常情况,特定的异常应能缩小可能受影响的止血成分范围,理想情况下简化后续检查。对于出现出血症状的患者,全面的病史和体格检查对于首先确定出血为病理性,然后在排除可识别病因后进行适当的诊断评估至关重要。了解任何特定出血性疾病中导致止血功能受损的病理生理过程可确保正确治疗,并避免不必要甚至禁忌的治疗方法。治疗方案还取决于出血表型,对于侵入性操作,则取决于预期的出血风险。咨询血液科医生可能有助于进行适当的评估和治疗,尤其是对于患有罕见出血性疾病或出血原因不明的成年人。本文综述了成人血液学实验室异常和异常出血的诊断方法,以及成人出血性疾病的基本预防保健和止血管理。

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