Dégot T, Métivier A -C, Casnedi S, Chenard M -P, Kessler R
Service de pneumologie, pôle de pathologie thoracique, nouvel hôpital Civil, CHU de Strasbourg, 67091 Strasbourg cedex, France.
Rev Pneumol Clin. 2009 Apr;65(2):101-7. doi: 10.1016/j.pneumo.2008.12.004. Epub 2009 Apr 1.
Castleman's disease is a rare orphan disease. The prevalence is estimated at less than 1/100 000. Respirologists may encounter this disease when its thoracic manifestations occur.
The authors report two cases of Castleman's disease with two different thoracic involvements. The first patient was a 20-year-old man without a previous medical history. A chance chest X-ray revealed right basal opacity. A lung biopsy demonstrated giant lymph node polyclonal hyperplasia leading to the diagnosis of a thoracic form of Castleman's disease. Since the patient was completely symptom free, no treatment was proposed. The patient was stable after 10months of medical supervision. The second patient, a 34-year-old woman, had a medical history of myasthenia gravis, autoimmune thrombopenic purpura and haemolytic anaemia. Her general condition deteriorated and upper mediastinal enlargement was noted. A diagnosis of multicentric Castleman's disease was established by means of the biopsy of an axillary lymph node. As the symptoms persisted, she was treated by rituximab. The clinical response was dramatic.
The authors call to mind the difficult diagnostic features and therapeutic strategies of Castleman's disease, a rare disease which may involve the thorax.
卡斯尔曼病是一种罕见的孤儿病。据估计,其患病率低于十万分之一。当出现胸部表现时,呼吸科医生可能会遇到这种疾病。
作者报告了两例卡斯尔曼病,胸部受累情况不同。首例患者为一名20岁男性,既往无病史。一次偶然的胸部X光检查发现右肺底部有阴影。肺活检显示巨大淋巴结多克隆增生,从而诊断为胸部型卡斯尔曼病。由于患者完全无症状,未建议进行治疗。经过10个月的医学监测,患者情况稳定。第二例患者为一名34岁女性,有重症肌无力、自身免疫性血小板减少性紫癜和溶血性贫血病史。她的一般状况恶化,发现上纵隔增宽。通过腋窝淋巴结活检确诊为多中心卡斯尔曼病。由于症状持续存在,她接受了利妥昔单抗治疗。临床反应显著。
作者提醒人们注意卡斯尔曼病这一可能累及胸部的罕见疾病的诊断难点和治疗策略。