tailieunhanh - Báo cáo khoa học: "A rare case of “switch on and off” multi-system Langerhans cell histiocytosis in an adult patient"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: A rare case of “switch on and off” multi-system Langerhans cell histiocytosis in an adult patient. | Karpathiou et al. Journal of Medical Case Reports 2011 5 302 http content 5 1 302 JOURNALOF medical case reports CASE REPORT Open Access A rare case of switch on and off multi-system Langerhans cell histiocytosis in an adult patient Georgia Karpathiou Anastasios Koutsopoulos and Marios E Froudarakis Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a switch on and off mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time no therapy or smoking cessation was indicated. Four years later she presented with bilateral pneumothorax and pulmonary lesions of Langerhans cell histiocytosis. She had pleurodesis for this disease-related complication and no further systemic treatment was applied except with regard to smoking cessation. During the follow-up period her pulmonary lesions regressed without recurrence six years after the initial lung involvement. Conclusion This uncommon case of remission of multi-system Langerhans cell histiocytosis indicates the unpredictable evolution of the disease raising the question of conservative management in such a patient. Introduction Langerhans cell histiocytosis LCH is a rare clinico-pathologic entity of unknown etiology with variable system involvement but with the common characteristic of sizable Langerhans cell infiltration 1 . The clinical course as well as the treatment and prognosis of this disease are not clearly identified especially in the adult population 2 . In pulmonary LCH smoking cessation is mandatory while glucocorticoid therapy despite being used has not been proved to be an effective treatment method 3 . In multi-system LCH children with