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2019•Tuberkuloz ve ToraksOpen access

Nadir bir hastalık; spontan pnömotoraks ile başvuran Erdheim-Chester hastalığı

Mustafa Buğra Coşkuner, Tevfik Özlü, Yılmaz Bülbül

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Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, it is characterized by organ infiltration of foamy histiocytes. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically CD68+ CD1a-. These characteristic histiocytes may be found in almost any tissue in cases of ECD. We present an interesting case of ECD that was presented by spontaneous pneumothorax and neurological involvement.

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Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, it is characterized by organ infiltration of foamy histiocytes. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically CD68+ CD1a-. These characteristic histiocytes may be found in almost any tissue in cases of ECD. We present an interesting case of ECD that was presented by spontaneous pneumothorax and neurological involvement.

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Available abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, it is characterized by organ infiltration of foamy histiocytes. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically CD68+ CD1a-. These characteristic histiocytes may be found in almost any tissue in cases of ECD. We present an interesting case of ECD that was presented by spontaneous pneumothorax and neurological involvement.

Key concepts: Erdheim–Chester disease, Histiocyte, Histiocytosis, Medicine, CD68, Pathology, Etiology, Infiltration (HVAC)

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