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Year : 2003  |  Volume : 13  |  Issue : 3  |  Page : 275-276
Pulmonary langerhan's cell histiocytosis X : A case report.


Department of Radiology, Amrita Institute of Medical Sciences, Amrita Lane, Elamakkara, Kochi-682 026, India

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Keywords: Langerhan′s cell histiocytosis, Hand-schuller Christian syndrome, Letterer-Siwe disease, Eosinophilic granuloma.

How to cite this article:
Dayananda L, Moorthy S, Prabhu N K, Sreekumar K P, Ghongade D V. Pulmonary langerhan's cell histiocytosis X : A case report. Indian J Radiol Imaging 2003;13:275-6

How to cite this URL:
Dayananda L, Moorthy S, Prabhu N K, Sreekumar K P, Ghongade D V. Pulmonary langerhan's cell histiocytosis X : A case report. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Feb 19];13:275-6. Available from: http://www.ijri.org/text.asp?2003/13/3/275/28696

   Introduction Top


Pulmonary Langerhan's Histiocytosis X is part of group of diseases of unknown etiology with extremely variable clinical manifestation and prognosis, but sharing the infiltration of involved tissue by large number of Langerhan's cell, often organized in granuloma.

We would like to report this rare case, which has typical radiological features.


   Case report Top


A 14-year-old male presented with anorexia, cough, and weight loss, on and off fever of 3-4 month duration and history of successful closure of PDA two years back. Physical examination was unremarkable except for emaciation. Routine hematology showed moderate leucocytosis (27u/l), neutrophilia (97%), normal eosenophilic count and raised ESR (50 mm at end of first hour). Frontal chest radiograph showed predominantly micro nodular shadows evenly distributed in all lung zones [Figure - 1]. Miliary tuberculosis was considered and patient was empirically started on anti tubercular drugs. Few days later patient was readmitted with worsening symptoms. Chest radiograph showed pneumothorax and reticulonodular shadows, predominantly involving the upper and mid zone [Figure - 2]. There was no evidence of volume loss. HRCT chest showed multiple thin walled cysts of varying size and shape unevenly distributed on a background of micro nodular lesions [Figure - 3]. Basal portion of lung were relatively spared. The findings were typical of Pulmonary Langerhan's Histiocytosis X. Whole body skeletal scitigraphy with 99 Tc-MDP did not reveal any bony lesion. Lung biopsy was advised but it was deferred since patient was not willing.


   Discussion Top


Langerhan's cell histiocytosis is a rare disorder with incidence of 10 cases per 2 million population as a whole. It consists of three clinico pathological entities.

  1. Hand-schuller Christian disease (multi focal Langerhan's cell histiocytosis)
  2. Letterer -Siwe disease (acute disseminated Langerhan's cell histiocytosis)
  3. Eosinophilic granuloma (unifocal Langerhan's cell histiocytosis)


Annual incidence of Pulmonary Langerhan's Histiocytosis X is estimated to be 1-2 cases per million population. It constitutes 3% of interstitial lung disease. There is no sex predilection. Peak age incidence is between 20-40 years. Smoking is a predisposing factor. Patient may present with pulmonary or systemic symptoms or an asymptomatic radiographic abnormality. Pneumothorax is a classical manifestation of Pulmonary Langerhan's Histiocytosis. Pneumothorax may be the initial manifestation in as high as 17% of patients. Respiratory function tests may show restrictive lung pattern and decreased carbon monoxide diffusion. Routine laboratory tests may show moderate leucocytosis. ESR is only occasionally raised. Chest radiograph commonly shows reticulonodular shadows. When cystic lesions are also seen, this constellation of findings is highly suggestive of Pulmonary Langerhan's Histiocytosis. In contrast to other interstitial lung disease (with the exception of lymphangioleiomatosis) lung volume are either normal or increased. On HRCT, lesions are evenly distributed between central and peripheral portions of the parenchyma. The basal portions of lung are relatively spared. Initially, nodular lesions predominate and some of them can cavitate. Nodules have centrilobular distribution. In a majority of patients nodules are associated with thick or thin walled cystic lesions and this pattern is highly suggestive of Histiocytosis X. As the disease evolves, cystic lesions become the predominant finding. Cysts are variable in size, usually less than 1 cm and may be confluent. When confluenting cysts are extensive, it gives the lung an emphysematous appearance. Pleural effusion and lymphadenopathy are extremely rare. Biopsy is indicated in cases where HRCT is inconclusive.The main differential diagnoses of predominantly cystic Pulmonary Langerhan's Histiocytosis X on HRCT are lymphangioleiomatosis and miliary tuberculosis. The most useful differtiating features are

  1. The cysts are distributed widely in Pulmonary Langerhan's Histioytosis X with relative sparing of lower zones. The cysts are evenly distributed in Lymphangioleiomatosis.
  2. Nodules are the hallmark of Pulmonary Langerhan's Histiocytosis X, while they are rare in Lymphangioleiomatosis.
  3. The cysts in Lymphangioleiomatosis tends to be uniformly round. In Pulmonary Langerhan's Histiocytosis X, cysts are of varying shape. They may be partially /completely septated or lobulated.
  4. Lymphangioleiomatosis is a disease seen only in females.
  5. Lymph node involvement and pleural effusions are common in Lymphangioleiomatosis.
  6. The presence of cysts helps to differentiate this condition from miliary tuberculosis[6].


 
   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 Langerhan's Cell Histiocytosis: prevalence, 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    
6.Alan G. Wilson, Peter Armstrong, Paul Dee, David M.Hansell.Imaging of Disease of the Chest 2000. 655-660   Back to cited text no. 6    

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Correspondence Address:
L Dayananda
Department of Radiology, Amrita Institute of Medical Sciences, Amrita Lane, Elamakkara, Kochi-682 026
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3]



 

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