摘要
血友病为凝血因子VIII或IX缺乏导致的遗传性疾病。血友病性假肿瘤是其罕见但严重的并发症,主要表现为关节外骨或软组织反复出血导致间室内积血,持续增大并压迫及破坏临近组织。临床上诊断主要根据患者外伤出血史及X线、超声波、CT和MRI等检查,其中MRI对诊断该病具有重要意义。对该病的治疗取决于假瘤的位置、大小、生长的速度及对周围组织的影响。治疗方式包括因子替代疗法和在此基础上的放疗及手术治疗(局部抽吸/引流、手术切除、截肢)等。但由于病情复杂常需个性化治疗。越早期获得明确诊断和治疗的患者预后越佳,因此早期准确的诊断和及时有效的治疗具有重要意义。
Haemophilia is a hereditary disease with factor VIII or IX deficiency. Haemophilic pseudotumour is a rare but se- rious complication seen in hemophiliacs. Progressive enlargement of a pseudotumor exerts increasing amounts of pressure on adja- cent structures due to recurrent extraarticular hemorrhage in either bone or soft tissues. Bones may be completely destroyed, and muscles and skin are often compressed. The diagnosis of haemophilicpseudotumouris based on the history of trauma and the ima- ging exaximation including X - ray, ultrasonography, CT scan and MRI （ the most important one） . Management depends on the site, size, rate of growth, and effect on adjoining structures. Options including factor replacement, radiotherapy and surgi- cal treatment （aspiration, surgical excisions and limb amputations）, may be necessary for pseudotumours. Actually, treatment is eventually rely on a careful case - to - case evaluation. Moreover, early diagnosis and adequate therapy of intramuscular hae- matomas should stop the development of haemophilic pseudotumours and help to improve the patients＇ quality of life.
出处
《中国矫形外科杂志》
CAS
CSCD
北大核心
2015年第5期426-430,共5页
Orthopedic Journal of China
关键词
血友病性假肿瘤
诊断
治疗
haemophilic pseudotumour
diagnosis
management
