Yasemin Çiğdem ÖZERDEM, Bahar MÜEZZİNOĞLU, Gür AKANSEL, Muhip KANKO, HÜSNÜ EFENDİ

Cardiac and cerebral involvement in Erdheim-Chester disease

Erdheim-Chester hastalığı (ECD) etyolojisi bilinmeyen, multipl organ tutulumu ile seyreden, sistemik histiyositozisin nadir bir non-Langerhans formudur. Dokularda CD68 ile pozitif ve CD1a ile negatif boyanma gösteren ksantomatöz infiltrasyon ile karekterizedir. Bu makalede 62 yaşında kötü koku alma şikayeti ile başvuran ve beyin manyetik rezonans görüntüleme tetkikinde klivusta kitle ve leptomeningeal tutulum saptanan ve eşlik eden intrakardiyak kitle eksizyonu sonucu histopatolojik tanısı ECD ile uyumlu bulunan bir olgu bildirilmektedir. Olgunun sıra dışı belirtileri, histopatolojik ve radyodiagnostik bulguları eşliğinde sunulmakta ve bu nadir hastalığın ayırıcı tanısı tartışılmaktadır.

Kalp ve beyin tutulumlu Erdheim-Chester hastalığı

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of systemic histiocytosis of unknown etiology that progresses with multiple organ involvement. It is characterized by a xanthomatous infiltration showing positive staining with CD68 and negative staining with CD1a in tissues. We report a 62 year-old case complaining of malodor, in the brain magnetic resonance imaging of whom a mass was found at the clivus and leptomeningeal involvement was determined. The accompanying intracardiac mass was excised and the case was diagnosed histopathologically as ECD. We present the unusual clinical symptoms along with histopathologic and radiodiagnostic findings and discuss the differential diagnosis of this rare disease

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