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Journals / Tüberküloz ve Toraks / 2019 / Cilt: 67 - Sayı: 3

A rare disease; Erdheim-Chester disease presenting with spontaneous pneumothorax

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

Mustafa Buğra COŞKUNER, Tevfik ÖZLÜ, Yılmaz BÜLBÜL

Pages
225–230
DOI
—

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.

Özet

Erdheim-Chester hastalığı (ECD) sebebi bilinmeyen köpüksü histiyositlerinorgan tutulumu ile karakterize non-Langerhans hücreli histiyositozis formudur. ECD tanısı doku biyopsilerinde tipik olarak CD68+ CD1a- histiyositlerintespitini içerir. Bu karakteristik histiyositler ECD olgularında hemen her dokuda bulunabilir. Spontan pnömotoraks ve nörolojik tutulum ile başvuran ilginçbir ECD olgusunu sunuyoruz.