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Year : 2000  |  Volume : 10  |  Issue : 2  |  Page : 115-116
Pulmonary histiocytosis - A case report with review of literature

Mandakini Imaging Centre, 90 Feet Road, Ghatkopar (E), Mumbai 400 077, India

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How to cite this article:
Parihar AL, Mashankar AS, Dialani VM, Salkade PR. Pulmonary histiocytosis - A case report with review of literature. Indian J Radiol Imaging 2000;10:115-6

How to cite this URL:
Parihar AL, Mashankar AS, Dialani VM, Salkade PR. Pulmonary histiocytosis - A case report with review of literature. Indian J Radiol Imaging [serial online] 2000 [cited 2021 Feb 26];10:115-6. Available from:

A twelve-year old boy presented with nonproductive cough and fever for four months and progressive breathlessness for two to three days.

The initial chest radiograph obtained on presentation showed bilaterally scattered multiple nodular opacities. The patient was treated with antibiotics, but there was no response. The last radiograph showed multiple cysts five to ten mm in diameter and a few tiny nodules with bilateral pneumothoraces.

Clinical examination revealed no evidence of lymphadenopathy or hepatosplenomegaly. High resolution CT (HRCT) of the chest performed with two mm thick scans revealed multiple cystic lesions distributed unevenly in both lungs along with multiple small nodules. Air space opacification was seen in the left upper lobe with bilateral large pneumothoraces [Figure - 1]. The costophrenic (CP) angles were not involved on either side. Thoracoscopic biopsy yielded the diagnosis of pulmonary histiocytosis. The patient was treated with bilateral pleurodesis. Pulmonary histiocytosis is a form of Langerhans cell histiocytosis. The main pathological feature of pulmonary histiocytosis is peribronchiolar inflammation, leading to fibrosis and cyst formation [1]. It is characterized by an abnormal proliferation of histiocytes, mixed with eosinophils, lymphocytes, plasma cells and neutrophils [2]. The clinical features include tachypnea, chronic or persistent cough, chest wall retraction and tracheal tug in children with systemic symptoms of fever, weight loss and fatigue [4].

Results of pulmonary function tests are non-specific and may show a mixed pattern with obstructive and restrictive components [2]. Pulmonary eosinophilic granuloma may be diagnosed by transbronchial biopsy or bronchoalveolar lavage, but definitive diagnosis often requires open or thoracic lung biopsy [2].

Plain radiograph findings are of preserved lung volumes with diffuse cysts and nodules, more common in the upper lobes sparing the costophrenic angles. HRCT is far superior to radiographs in the diagnosis of the upper lobe nodules and cysts with relative sparing of bases, which are virtually pathognomonic of this disorder [2]. Nodules upto ten mm are frequently seen on presentation and tend to decrease in profusion, with cysts becoming a more prominent feature as the disease progresses [1],[2]. The cysts may vary in size from one to ten mm. The wall thickness of the cyst may vary, mimicking bronchiectasis or areas of emphysema.

Parenchymal opacification may be seen, which is due to a desquamative interstitial pneumonia like reaction [3]. End-stage lung disease is diagnosed by the presence of honeycombing, extensive cystic changes or conglomerate fibrosis. Mediastinal adenopathy is more common in children [2]. About 30% of patients have constitutional symptoms but about 25% are asymptomatic [2].

Isolated lung disease in histiocytosis is most common in adults [2]. Upper zonal prominence is the most common [2]. Spontaneous pneumothoraces are identified on presentation in 25% of patients [2]. Pleural effusion and pleural thickening are almost never seen [2].

This disease seems to have a favorable prognosis with a high rate of spontaneous remission [5]. Cessation of smoking is the first line of treatment. Corticosteroids are added if the disease progresses. In some cases cytotoxic drugs have been used. Pleurodesis may be necessary for recurrent pneumothoraces.

   References Top

1.Kulwiec EL, Lynch DA, Aguayo SM, Shwarz MJ, King TE Jr. Imaging of pulmonary histiocytosis. Radiographics 1992; 12: 515-526.   Back to cited text no. 1    
2.Gervais DA, Whitman GJ, Chew FS, Pulmonary eosinophilic granuloma. AJR 1993; 161: 1158   Back to cited text no. 2    
3.Leung AN, Miller RR, Muller NL Parenchymal opacification in chronic infiltrative lung diseases: CT - pathologic correlation. Radiology 1993;188: 209-214.   Back to cited text no. 3    
4.Ha SY, Helms P, Pletcher M et al . Lung involvement in Langerhans Cell Histiocytosis: prevalance, CT features and outcome. Pediatrics 1992; 89: 446-469.   Back to cited text no. 4    
5.Friedman PJ, Liebow AA, Sokoloft J. Eosinophilic granuloma of lung. Clinical aspects of primary pulmonary histiocytosis in adults. Medicine 1981; 60: 385-396.  Back to cited text no. 5    

Correspondence Address:
Anand L Parihar
Mandakini Imaging Centre, 90 Feet Road, Ghatkopar (E), Mumbai 400 077
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Source of Support: None, Conflict of Interest: None

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[Figure - 1]

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