Akçay S, Eyüboglu F O, Arícan A, Demírhan B
Department of Pulmonary Diseases, Başkent University Hospital, Fevzi Cakmak Bulvari 10 Sokak, No: 45 06490 Bahçelievler, Ankara, Turkey.
Respirology. 2001 Dec;6(4):357-60. doi: 10.1046/j.1440-1843.2001.00353.x.
Langerhans' cell histiocytosis (LCH) is a condition in which granulomas form in various tissues through the accumulation of abnormal histiocytes (Langerhans' cells), granulocytes and lymphocytes. An important distinction between LCH and primary pulmonary histiocytosis (PPH) is that smoking cessation is known to often lead to spontaneous improvement in PPH patients, while this has not been demonstrated in patients with multisystem LCH. In this case report, we describe the case of a 20-year-old man who presented with cough and palpably enlarged lymph nodes in his neck. An inguinal lymph node biopsy led to the diagnosis of multisystem LCH in the lungs, the cervical, intra-abdominal, and inguinal lymph nodes and the spleen. The patient was a smoker, and had averaged 30 cigarettes per day for 7 years. To address the multisystem involvement, intravenous pulse steroid therapy was administered (30-40 mg/kg daily) in two 3-day treatment periods separated by 3-4 weeks. The patient was also advised to stop smoking, and did so. Serial follow-up examinations confirmed an objective improvement with complete remission. The patient was still healthy at 18 months post-treatment, reflecting a dramatic and positive response to pulse steroid therapy. This encouraging result makes this case an important one to highlight.
朗格汉斯细胞组织细胞增多症（LCH）是一种通过异常组织细胞（朗格汉斯细胞）、粒细胞和淋巴细胞的积聚在各种组织中形成肉芽肿的病症。LCH与原发性肺组织细胞增多症（PPH）的一个重要区别在于，已知戒烟通常会使PPH患者自发改善，而多系统LCH患者尚未证实这一点。在本病例报告中，我们描述了一名20岁男性患者，他出现咳嗽且颈部淋巴结可触及肿大。腹股沟淋巴结活检确诊为肺部、颈部、腹腔和腹股沟淋巴结以及脾脏的多系统LCH。该患者为吸烟者，7年来平均每天吸30支烟。为解决多系统受累问题，在相隔3 - 4周的两个3天治疗期内给予静脉脉冲类固醇治疗（每日30 - 40 mg/kg）。患者还被建议戒烟，并且他做到了。系列随访检查证实有客观改善且完全缓解。治疗后18个月患者仍健康，这反映出对脉冲类固醇治疗有显著且积极的反应。这一令人鼓舞的结果使该病例成为一个值得强调的重要病例。
