摘要
目的提高对淋巴管平滑肌瘤病(LAM病)的认识。方法对我院近2年诊断的4例淋巴管平滑肌瘤病临床资料进行分析。结果LAM病是一种原因不明,通常累及育龄妇女的罕见疾病,其可散发或与结节性硬化同时存在,组织学以肺实质不典型平滑肌细胞增生为特征,临床上表现为进行性呼吸困难、乳糜性胸腹水、自发性气胸或错构瘤等。LAM病治疗困难,预后较差。结论临床医生应掌握此病的特点,尽早诊断及治疗,以改善预后。
Objective To improve the diagnosis and treatment of lymphangioleiomyomatosis(LAM). Methods The clinical data of four cases of lymphangioleiomyomatosis diagnosed in our hospital were analyzed and corresponding literature was reviewed. Results Lymphangioleiomyomatosis, a rare disorder of unknown cause that occured almost exclusively in women of child bearing ages, was characterized microscopically by atypical smooth muscle proliferation. It could occur as a sporadic diseases or as a part of tuberous sclerosis complex(TSC). LAM was associated with progressive dyspnea, recurrent spontaneous pneumo-thoraces, chylous effusions, and hamartomas. Conclusion Clinicians should be familiar with the char-acteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.
出处
《中国医学科学院学报》
CAS
CSCD
北大核心
2004年第3期306-309,i006,共5页
Acta Academiae Medicinae Sinicae
关键词
淋巴管平滑肌瘤病
平滑肌增生
lymphangioleiomyomatosis
smooth muscle proliferation
