Department of Surgery, University of Virginia, Charlottesville, Virginia 22908, USA.
Ann Thorac Surg. 2010 Jan;89(1):300-2. doi: 10.1016/j.athoracsur.2009.05.016.
Rosai-Dorfman disease is rare and typically presents with cervical lymphadenopathy, but may manifest as extranodal disease. This disease is generally indolent and self-limited, but it carries a poor or fatal prognosis when it is advanced or when it involves and compresses vital structures. We present a case of Rosai-Dorfman disease affecting the pulmonary arteries in a 22-year-old woman with severe, symptomatic right heart failure.
罗萨达-多夫曼病较为罕见,通常表现为颈淋巴结病,但也可能表现为结外疾病。该疾病通常为惰性和自限性的,但当疾病进展或累及并压迫重要结构时,预后较差或危及生命。我们报告了一例 22 岁女性患有罗萨达-多夫曼病,累及肺动脉,导致严重的、有症状的右心衰竭。