Ersin GÜNAY, Yetkin AĞAÇKIRAN, Selma GÜVEN FIRAT, Zafer AKTAŞ, Hakan ERTÜRK, Tuğrul ŞİPİT

Pulmonary involvement in sea-blue histiocytosis

Sea-blue histiyositoz Niemann-Pick hastalığının altı tipinden biridir. Çocukluk çağında başlayan hepatosplenomegali olma-sı, nörolojik tutulumun olmaması ve sfingomiyelinaz aktivitesinin azalmasıyla karakterizedir. Akciğer tutulumu sea-bluehistiyositozda görülen nadir bir klinik tutulumdur. Bu olgu sunumunda, 15 yaşındayken sea-blue histiyositoz tanısı konu-lan 39 yaşındaki bir erkek hasta sunulmaktadır. Hasta kliniğimize prodüktif öksürük, hemoptizi, ateş ve kilo kaybıyla baş-vurdu. Hastanın semptomlarında antibiyotik tedavisine rağmen gerileme olmaması üzerine ileri araştırma sonucunda sea-blue histiyositozun akciğer tutulumu olduğu görüldü. İlginç olarak, hastanın yakınmaları tanısal bronkoalveoler lavaj son-rası düzeldi. Nadir görülmesi nedeniyle hasta literatür eşliğinde tartışıldı.

Sea-blue histiyositoz akciğer tutulumu

Sea-blue histiocytosis is one of the six types of Niemann-Pick disease. It is characterized by childhood onset of hepatosple-nomegaly, lack of neurological involvement and diminished sphingomyelinase activity. Pulmonary system is rarely invol-ved sea-blue histiocytosis. In this paper, we present a 39-years-old male who had previously diagnosed as sea-blue histi-ocytosis at the age of 15. He was admitted to our clinic due to productive cough, hemoptysis, fever and weight loss. Hissymptoms did not resolve with the antibiotic treatment and further investigations revealed pulmonary involvement of sea-blue histiocytosis. After diagnostic bronchoalveolar lavage, his symptomes were improved, interestingly. This rare entitywas discussed with literature survey.

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