RESUMO
BACKGROUND: Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. METHOD: Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. RESULT: There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. CONCLUSION: In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.
Assuntos
Feminino , Humanos , Masculino , Dor no Peito , Tosse , Tratamento Farmacológico , Dispneia , Seguimentos , Histiocitose , Histiocitose de Células de Langerhans , Células de Langerhans , Fígado , Pulmão , Doenças Pulmonares Intersticiais , Linfonodos , Mortalidade , Hipófise , Pneumotórax , Prognóstico , Radiografia , Estudos Retrospectivos , Seul , Pele , Tórax , Glândula TireoideRESUMO
Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and , occaionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.
Assuntos
Humanos , Adulto Jovem , Biópsia , Encéfalo , Dor no Peito , Tosse , Citoplasma , Desamino Arginina Vasopressina , Diabetes Insípido , Diabetes Insípido Neurogênico , Dispneia , Histiocitose de Células de Langerhans , Células de Langerhans , Pulmão , Imageamento por Ressonância Magnética , Pneumotórax , Polidipsia , Poliúria , Proteínas S100 , Abandono do Hábito de Fumar , Tórax , Privação de Água , Filme para Raios XRESUMO
Pulmonary histiocytosis X is an idiopathic benign disease characterized by proliferation and infiltration of lung tissue by characteristic Langerhans cells and eosinophils. Pulmonary histiocytosis X is common in young male adults, and shows variable clinical characteristics. We experienced a case of pathologically proven pulmonary histiocytosis X in a 30-year-old man who visit to our hospital due to chest discomfort and cough. The chest radiograph of our patient shows right pneumothorax and characteristic multiple thin-walled cysts on the both upper lung fields. The HRCT shows multiple thin-walled cysts, a few scattered nodules in both upper and right middle lung, and right pneumothorax.
Assuntos
Adulto , Humanos , Masculino , Tosse , Eosinófilos , Histiocitose de Células de Langerhans , Células de Langerhans , Pulmão , Pneumotórax , Radiografia Torácica , TóraxRESUMO
Histiocytosis-X is a term used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the Pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilateral, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X which is confined by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.