Pulmonary involvement in sea-blue histiocytosis

SUMMARY Pulmonary involvement in sea-blue histiocytosis Ersin GUNAY 1 , Selma FIRAT GUVEN 1 , Zafer AKTAS 1 , Tugrul SIPIT 1 , Yetkin AĞACKIRAN 2 , Hakan ERTURK 3 1 Clinic of Chest Diseases, Ataturk Chest Diseases and Chest Surgery Training and Research Hospital, Ankara, Turkey, 2 Clinic of Pathology, Ataturk Chest Diseases and Chest Surgery Training and Research Hospital, Ankara, Turkey, 3 Clinic of Radiology, Ataturk Chest Diseases and Chest Surgery Training and Research Hospital, Ankara, Turkey. 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.