Pneumothorax in Patients with Pulmonary Langerhans Cell Histiocytosis.

INTRODUCTION: Pneumothorax often develops in pulmonary Langerhans cell histiocytosis (PLCH), but some patients take a long time to be correctly diagnosed. OBJECTIVES: This study assessed the frequency of pneumothorax in PLCH and analysed the role of chest computed tomography (CT) in the prompt diagnosis. PATIENTS AND MATERIAL: Of the 90 patients with PLCH seen from 2000 to 2015, 29 (32%) had pneumothorax as the initial finding. In this group, 18 (62%) patients were diagnosed within 1 month, whereas the diagnosis was delayed for 4-120 months in 11 (38%) patients. RESULTS: Patients who had pneumothorax as the initial sign of PLCH tended to be younger (mean age 27.7 ± 7.92 vs. 39.9 ± 13.21 years; P = 0.0001), male (69% vs. 43%; P = 0.028), smoked less (mean pack/years 8.4 ± 6.85 vs. 19 ± 17.16; P = 0.003), and had a significantly lower mean FVC (77.96 ± 19.62 vs. 89.47 ± 21.86% pred.; P = 0.015) and FEV1 (68.6 ± 19.93 vs. 79.4 ± 21.48% pred.; P = 0.03 than patients who had no pneumothorax. Recurrent pneumothorax was diagnosed more frequently in the group with a delayed diagnosis (82% vs. 39%; P = 0.02). CT was performed in all of the patients who were diagnosed promptly, but in none of the patients with a delayed diagnosis. CONCLUSIONS: Patients who had pneumothorax as the initial sign of PLCH were younger, more frequently men, and had greater respiratory impairment than those who had no pneumothorax. CT in patients with pneumothorax led to a correct diagnosis of this disease.

The value of the new scoring system for predicting neoplastic pericarditis in the patients with large pericardial effusion.

PURPOSE: Early recognition of neoplastic pericarditis (npe) is crucial for the planning of subsequent therapy. The aim of the present study was to construct the scoring system assessing the probability of npe, in the patients requiring pericardial fluid (pf) drainage due to large pericardial effusion. METHODS: One hundred forty-six patients, 74 males and 72 females, entered the study. Npe based on positive pf cytology and/or pericardial biopsy specimen was recognised in 66 patients, non-npe in 80. Original scoring system was constructed based on parameters with the highest diagnostic value: mediastinal lymphadenopathy on chest CT scan, increased concentration of tumour markers (cytokeratin 19 fragments-Cyfra 21-1 and carcinoembryonic antigen-CEA) in pf, bloody character of pf, signs of imminent cardiac tamponade on echocardiography and tachycardia exceeding 90 beats/min on ECG. Each parameter was scored with positive or negative points depending on the positive and negative predictive values (PPV, NPV). RESULTS: The area under curve (AUC) for the scoring system was 0.926 (95%CI 0.852-0.963) and it was higher than AUC for Cyfra 21-1 0.789 (95%CI 0.684-0.893) or CEA 0.758 (95%CI 0.652-0.864). The score optimally discriminating between npe and non-npe was 0 points (sensitivity 0.84, specificity 0.91, PPV 0.9, NPV 0.85). CONCLUSION: Despite chest CT and tumour marker evaluation in pericardial fluid were good discriminators between npe and non-npe, the applied scoring system further improved the predicting of neoplastic disease in the studied population.