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Pediatric Interstitial Lung Diseases
Langerhans Cell Histiocytosis, Pulmonary
Langerhans Cell Histiocytosis, Pulmonary
Randy R. Richardson, MD; Mantosh S. Rattan, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, histiocytosis X

            • Definitions

              • Langerhans cell histiocytosis (LCH): Neoplastic proliferation of monoclonal Langerhans cells in 1 or more organ systems
                • Leads to formation of destructive granulomas

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Della Valle V et al: Chest computed tomography findings for a cohort of children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(10):e28496, 2020
                                  2. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                  3. Le Louet S et al: Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study. Orphanet J Rare Dis. 15(1):241, 2020
                                  4. Allen CE et al: Langerhans-Cell histiocytosis. N Engl J Med. 379(9):856-68, 2018
                                  5. Jezierska M et al: Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol. 35(1):6-17, 2018
                                  6. Retraction: 'Imaging characteristics of liver Langerhans cell histiocytosis in children' by Hui Liu, Xueying Long, Xiaoyi Wang, Wenzheng Li and Yigang Pei. Pediatr Int. 59(9):1034, 2017
                                  7. Ahuja J et al: Histiocytic disorders of the chest: imaging findings. Radiographics. 35(2):357-70, 2015
                                  8. Bano S et al: Pulmonary Langerhans cell histiocytosis in children: a spectrum of radiologic findings. Eur J Radiol. 83(1):47-56, 2014
                                  9. Ronceray L et al: Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr. 161(1):129-33.e1-3, 2012
                                  10. Seely JM et al: Pulmonary Langerhans cell histiocytosis: a comparative study of computed tomography in children and adults. J Thorac Imaging. 27(1):65-70, 2012
                                  11. Suri HS et al: Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 7:16, 2012
                                  12. García-Peña P et al: Thoracic findings of systemic diseases at high-resolution CT in children. Radiographics. 31(2):465-82, 2011
                                  13. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                  14. Odame I et al: Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer. 47(7):889-93, 2006
                                  15. Abbott GF et al: From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics. 24(3):821-41, 2004
                                  16. Donnelly LF et al: Langerhans' cell histiocytosis showing low-attenuation mediastinal mass and cystic lung disease. AJR Am J Roentgenol. 174(3):877-8, 2000
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Clinical Issues

                                          • Diagnostic Checklist

                                            TERMINOLOGY

                                            • Synonyms

                                              • Eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, histiocytosis X

                                            • Definitions

                                              • Langerhans cell histiocytosis (LCH): Neoplastic proliferation of monoclonal Langerhans cells in 1 or more organ systems
                                                • Leads to formation of destructive granulomas

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Staging, Grading, & Classification

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Della Valle V et al: Chest computed tomography findings for a cohort of children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(10):e28496, 2020
                                                                  2. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                                                  3. Le Louet S et al: Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study. Orphanet J Rare Dis. 15(1):241, 2020
                                                                  4. Allen CE et al: Langerhans-Cell histiocytosis. N Engl J Med. 379(9):856-68, 2018
                                                                  5. Jezierska M et al: Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol. 35(1):6-17, 2018
                                                                  6. Retraction: 'Imaging characteristics of liver Langerhans cell histiocytosis in children' by Hui Liu, Xueying Long, Xiaoyi Wang, Wenzheng Li and Yigang Pei. Pediatr Int. 59(9):1034, 2017
                                                                  7. Ahuja J et al: Histiocytic disorders of the chest: imaging findings. Radiographics. 35(2):357-70, 2015
                                                                  8. Bano S et al: Pulmonary Langerhans cell histiocytosis in children: a spectrum of radiologic findings. Eur J Radiol. 83(1):47-56, 2014
                                                                  9. Ronceray L et al: Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr. 161(1):129-33.e1-3, 2012
                                                                  10. Seely JM et al: Pulmonary Langerhans cell histiocytosis: a comparative study of computed tomography in children and adults. J Thorac Imaging. 27(1):65-70, 2012
                                                                  11. Suri HS et al: Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 7:16, 2012
                                                                  12. García-Peña P et al: Thoracic findings of systemic diseases at high-resolution CT in children. Radiographics. 31(2):465-82, 2011
                                                                  13. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                                                  14. Odame I et al: Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer. 47(7):889-93, 2006
                                                                  15. Abbott GF et al: From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics. 24(3):821-41, 2004
                                                                  16. Donnelly LF et al: Langerhans' cell histiocytosis showing low-attenuation mediastinal mass and cystic lung disease. AJR Am J Roentgenol. 174(3):877-8, 2000