کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3466125 1596545 2015 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Pulmonary Langerhans cell histiocytosis: A comprehensive analysis of 40 patients and literature review
ترجمه فارسی عنوان
هیستوسیتوز سلول لینگ ریه: تجزیه و تحلیل جامع از 40 بیمار و بررسی ادبیات
کلمات کلیدی
هیستوسیتوز سلول لانگرهانس ریه، مدیریت، مشارکت سیستمیک
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی پزشکی و دندانپزشکی (عمومی)
چکیده انگلیسی


• We conducted a retrospective analysis on clinical data of 40 patients with PLCH.
• PLCH might be considerate in young adult smokers with a typical radiological pattern.
• Early diagnosis and smoking cessation are associated with a good prognosis.

BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial disease affecting primarily young adult smokers. In order to highlight the clinical features of the disease, we conducted a retrospective analysis on clinical data of PLCH patients followed at our center; moreover, we reviewed the current literature on PLCH.Method and resultsBetween January 2004 and July 2014, 40 patients with PLCH were evaluated at our Division. The average patients' age was 40 (± 14) years, and 22 of them were females. Diagnosis was based on search of CD1a + cells in the bronchoalveolar lavage (10 patients), lung biopsy (8 patients), or cystic bone lesion's biopsy (2 patients); in 12 patients, diagnosis was achieved on the basis of the clinical-radiological data.The principal manifestation of PLCH was the presence of cysts involving upper lung zones with costophrenic sparing on chest CT scan (in 25 patients); micronodular pattern in the middle-upper zone and combination of the two radiological patterns were less frequently observed (in 9 and 6 patients, respectively). Pulmonary hypertension was found in 4 patients. Extra pulmonary manifestations were diabetes insipidus, bone lesions, and skin involvement (in 5, 7, and 1 patient, respectively).For 25 patients, smoking cessation was the only required therapy. Treatments with low dose of prednisolone, vinblastine and prednisolone, or 6-mercaptopurin were reserved for patients with major pulmonary or extra-pulmonary involvement (for 11, 4, and 5 patients, respectively).In conclusion, PLCH is a rare, multi-systemic disease; early diagnosis, accurate staging and smoking cessation are considered critical in PLCH management.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: European Journal of Internal Medicine - Volume 26, Issue 5, June 2015, Pages 351–356
نویسندگان
, , , , ,