Advanced Langerhans Cell Histiocytosis- A case report of a rare disease

Abstract

Adult Langerhans Cell Histiocytosis (LCH) is a quite rare and tobacco smoking related disease. We report on a case of pulmonary LCH, diagnosed at the Teaching Hospital of Lung Disease, Clinical Centre of Serbia in Belgrade. On admission, a 45-year-old mechanician, smoker (30 pack/years) had severe dyspnea, fatigue and massive pretibial/ ankle oedema due to right-heart failure. Since the age of 19 years, he suffered from recurrent pneumothorax, and the first radiographic changes (bilateral micronodular shadows) were present at the age of 39 together with obstructive disturbance of ventilation and respiratory failure. At the patient's age of 40 years, open lung biopsy led to diagnosis of LCH. Investigation was completed with computed tomography scans, hemodynamic investigation (heart catheterization and pneumoangiography), echocardiography, skeletal scintigraphy, and additional lung function testing. Treatment included methylprednisolon, sildenafil, and diuretics. The patient is being followed up and severe pulmonary arterial hypertension is usually considered a poor prognosis.