Over a 29-year period, 43 cases of histiocytosis X presented in children under the age of 12 years. 29 patients (67%) have survived, and of these, 15 (52%) have a detectable disability. It was confirmed that young age at presentation and evidence of soft tissue involvement were associated with a worse prognosis. The majority of deaths were associated with pulmonary involvement. 14 patients deve...

Malignant histiocytosis was first described in 1939 by Scott and Smith as histiocytic medullary reticulosis(1). We report a case of Histiocytosis X in a 2year-old by presenting with chronic discharging ears, which is a rare manifestation of the disease. A 2-year-old boy was admitted with complaints of chronic ear discharge and fever of 1 month duration. He had recurrent vague pain abdomen and l...

The occurrence together of two major distinct diseases in the oral area is relatively rare. In this report, a case is presented in which histiocytosis X was found to develop in a child with acute lymphoblastic leukemia. The nature of the differentiated histiocytoses is explored, and the possibility of the relationship between a lymphoproliferative and a histiocytic disease entity is investigated.

Langerhans’ cell histiocytosis may cause irreversible respiratory failure due to progressive destruction of lung parenchyma and widespread cystic change. Transplantation oVers a therapeutic option. A case is described of recurrence of Langerhans’ cell histiocytosis which was associated with deterioration in lung function four years following bilateral lung transplantation. Patients transplanted...

Histiocytosis X (HX) is a rare disease. There are many questions and debates in its etiology, pathology, clinical type and therapy. To enhance the recognition of otolaryngologists in HX of the ear and temporal bone and the level of diagnosis and therapy, 30 years' results of 21 patients with HX of the ear and temporal bone proved by histiopathology were reviewed, 18 patients were cured (85%), 6...

Langerhans cell histiocytosis (LCH) as a term encompasses eosinophilic granuloma and two clinical syndromes: Letterer-Siwe disease and Hand-Schüller-Christian. All these syndromes seem to represent similar processes in which the proliferating cells have the structural and functional features of Langerhans cells. In reviewing the literature found that this disease does indeed occur in some famil...

INTRODUCTION Adult pulmonary Langerhans'cell histiocytosis, also referred to as histiocytosis X, is a disorder of unknown etiology which affects preferentially young smokers. The disease is characterized by granulomatous lesions which progressively invade and destroy distal airways, leading to the formation of characteristic cicatricial kystic lesions. Florid granulomas contain numerous Langerh...