Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. High-resolution computed tomography of the chest (HRCT) plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, often forming 'tree in bud' lesions, cavitated nodules, thin-and thick-walled cystic lesions frequently confluent. Definite diagnosis requires the finding of characteristic lesions in histological examination and demonstration of antigen CD1a or CD207 presenting cells in immunohistochemistry. Smoking cessation is the most important recommendation for PLCH patients. There are no evidence based data regarding systemic steroid therapy. The treatment of progressive PLCH is based on cladribine or cytarabine as salvage therapy. The prognosis is good, and over 85% of patients survive 10 years.
The work was created within the statutory activity of the National Research Institute of Tuberulosis and Lung Diseases in Warsaw, Poland, research task nr 1.10 "Molecular epidemiological investigation among staphylococci isolated from patients with granulomatosis with polyangiitis".
Primary pulmonary angiosarcoma is an extremely rare disease. Chest computed tomography demonstrates solitary or multifocal lesions, sometimes associated with ground-glass opacities or pleural effusion. Diagnosis is based on histological examination that reveals spindle-shaped epithelioid cells with positive staining for endothelial markers (factor VIII, CD 31, CD34, Fli-1, Ulex europaeus agglutinin 1, vimentin). The prognosis is poor and effective treatment is still being researched. This is a report of a 65-year-old patient with a four-month history of haemoptysis, cough, and dyspnoea. The primary radiological findings suggested interstitial lung disease. After one month the clinical presentation evolved into diffuse pulmonary haemorrhage with concomitant haemothorax. The diagnosis of primary lung angiosarcoma was based on histological and immunohistochemical examination of the lung and pleural biopsy obtained by videothoracoscopy.
Promienica to rzadko występująca, przewlekła choroba infekcyjna wywołana przez bakterie beztlenowe Gram-dodatnie z rodzaju Actinomyces. Indukują one odczyn zapalny o charakterze ropnym w tkankach. Promieniowce są komensalami bytującymi w jamie ustnej, przewodzie pokarmowym i drogach rodnych, a infekcje mają charakter niemal wyłącznie endogenny. Ze względu na zmienny obraz kliniczny, przewlekły przebieg, częściej przypomina chorobę nowotworową niż zakaźną. Przedstawiamy przypadek 56-letniego mężczyzny z niezwykle rzadką płucną manifestacją promienicy w postaci obustronnych rozsianych guzków w miąższu płuc z objawami ogólnymi, po wstępnym leczeniu przeciwprątkowym. Rozpoznanie ostatecznie ustalono na podstawie badania histologicznego wycinków z płuc. Po 7 miesiącach leczenia przeciwbakteryjnego uzyskano poprawę kliniczną i radiologiczną.
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