engleski

Adult multi-organ Langerhans cell histiocytosis with fatal outcome

INTRODUCTION. Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with a Langerhans cell phenotype. It contains some of the elements of both a neoplastic process and a reactive immune disorder. CASE REPORT. A 55-year-old female patient presented in March 2014 with diarrhea, fever, leg edema and skin lesions on the torso. She was an ex-smoker who had been treated for COPD since 2011 and suffered from dermatitis-like skin lesions for the previous 5 years. Thoracic MSCT revealed a pathological reticular lung pattern. Langerhans cells (S-100+, CD1a+) where found in bioptic materials of epidermis, intestinal mucosa and bone marrow, whereas non-specific macrophage collections (CD68+, CD1a-) were found in lung tissue. Treatment with methylprednisolone resulted in significant clinical improvement. In November 2014, she developed a spontaneous left pneumothorax. A month later, she was diagnosed with active M.tuberculosis infection. The patient died one week after starting triple ATL therapy, in January 2015. CONCLUSION. LCH has various clinical manifestations which often lead to misdiagnosis or delayed diagnosis of this rare condition, especially in adults. Our patient had a multiple system disease with the involvement of "risk organs", predicting poor prognosis.

histiocytosis; lung; adult; Langerhans cell

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