engleski

Langerhans cell histiocytosis complicated with M. tuberculosis infection in adult patient: a case report

Background: Langerhans cell histiocytosis (LCH) encompasses a group of rare disorders characterized by proliferation of cells with a Langerhans cell phenotype. It is still debated whether it is a neoplastic process or a reactive immune disorder. In this paper we report a case of a female patient with adult-onset LCH, diagnosed and treated in CHC Rijeka 2014-2015. Case: A 55-year-old female patient was admitted to the Department of Pulmonology, CHC Rijeka in March 2014, after a transfer from a general hospital where she had been treated for dyarrhea, fever, leg edema and eczematous skin lesions of the torso. The MSCT of the thorax performed at the mentioned institution revealed a pathological reticular lung pattern. The patient was an ex-smoker, treated for COPD since 2011 and struggling with persistent dermatitis-like skin lesions for the previous 5 years. A thorough interdisciplinary diagnostic work-up including MR-scans and biopsies of several different tissues was conducted, discovering enlarged spleen, infiltrates of Langerhans cells (S-100, CD1a, CD68) in lung tissue, epidermis, intestinal mucosa and bone marrow. Treatment with methylprednisolone was started, leading to significant clinical improvement. In November 2014 she developed a spontaneous left pneumothorax. Follow up CXRs after a successful thoracic drainage revealed a progressive area of left lung consolidation, with signs of cavitation. Thoracic HR-MSCT was highly suggestive for active M. tuberculosis infection in the left lung upper lobe, together with the positive QFT (8.27) and typical symptoms of appetite loss, exhaustion and night sweating. Diffuse cistic nodules described in the rest of the lung parenchyma could've been a part of pulmonary LCH, however, miliary tuberculosis was also possible. One week after starting standard triple ATL therapy, the patient was admitted to hospital in hypovolemic shock due to severe dyarrhea, with respiratory insufficiency, hypoalbuminemia, anasarca, altered liver probes and coagulopathy. Despite all medical efforts, the patient died on the 13th day of the treatment, in January 2015, at the age of 56. The results of bronchial aspirate cultivation on MGIT and L-J arrived post-mortally -M.tuberculosis was isolated on both. Conclusion: LCH has various clinical manifestations which often leads to misdiagnosis or late diagnosis of this rare condition, especially in adults. Our patient had a multy-sistem disease with the involvement of „risk organs“, predicting poor prognosis. Her severe condition was further endangered with active tuberculosis infection, which consequently led to a fatal outcome.

Langerhans cell histiocytosis; Spontaneous pneumothorax; Tuberculosis

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