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New entity of an isolated pulmonary non-Langerhans cell histiocytosis

Michael Kreuter, Claus Peter Heussel, Christian Nagel, Richard P. Baum, Philipp A. Schnabel, Felix J.F. Herth, Dirk Theegarten
European Respiratory Journal 2013 42: 5005; DOI:
Michael Kreuter
1Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
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Claus Peter Heussel
2Clinical and Interventional Radiology, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
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Christian Nagel
1Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
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Richard P. Baum
3Zentrum für Molekulare Bildgebung, Zentralklinik Bad Berka, ENETS Center of Excellence, Bad Berka, Germany
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Philipp A. Schnabel
4Institute for Pathology, University of Heidelberg, Heidelberg, Germany
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Felix J.F. Herth
1Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
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Dirk Theegarten
5Institute for Pathology, University of Essen, Essen, Germany
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Abstract

Background: Histiocytic disorders are rare, heterogeneous diseases with abnormal tissue infiltration by histiocytes. So far, pulmonary manifestation in Non-Langerhans cell histiocytoses (NLCH) occurred only as a part of a systemic disease.

Case: A 37 year old male stonesculptor suffered from progressive dyspnea and thoracic tightness. He was admitted under suspicion of pulmonary capillary hemangiomatosis (PCH). Asthma was diagnosed 6 years ago and he had a former smoking history of 5 pack years. Pulmonary function testing demonstrated obstructive lung disease (FEV1 48%) with negative bronchospasmolysis and compromised diffusion capacity (DLCO 50%, KCO 54%). Cardiopulmonary exercise testing showed a maximum work load of 150 W with VO2peak 20 ml/min/kg (56%). Surgical lung biopsies excluded PCH and revealed interstitial histiocytic infiltrates. These showed immunohistochemical positivity for CD68 and negativity for CD1a and Langerin, therefore NLCH was diagnosed. CT and metabolic imaging with F-18 FDG PET/CT scanning -as used diagnosing manifestation in Erdheim-Chester disease- demonstrated bipulmonary, FDG-avid nodular infiltrates and excluded extrapulmonary manifestations. After multidisciplinary discussion a watch and wait strategy was chosen. The patient is currently stable since 18 months with regards to clinical, functional and radiological parameters.

Conclusion: Here we describe a new entity of an isolated pulmonary NLCH. Clinical, patho- & radiological features are not consistent with any other NLCH reported so far. The association with asthma might be coincidental. The etiopathogenesis is unclear and might however be occupational or smoking-related in this case. Long term prognosis seems to be dubious.

  • Interstitial lung disease
  • Chronic disease
  • Orphan disease
  • © 2013 ERS
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New entity of an isolated pulmonary non-Langerhans cell histiocytosis
Michael Kreuter, Claus Peter Heussel, Christian Nagel, Richard P. Baum, Philipp A. Schnabel, Felix J.F. Herth, Dirk Theegarten
European Respiratory Journal Sep 2013, 42 (Suppl 57) 5005;

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New entity of an isolated pulmonary non-Langerhans cell histiocytosis
Michael Kreuter, Claus Peter Heussel, Christian Nagel, Richard P. Baum, Philipp A. Schnabel, Felix J.F. Herth, Dirk Theegarten
European Respiratory Journal Sep 2013, 42 (Suppl 57) 5005;
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