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Langerhans Cell Histiocytosis
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease, Letterer-Siwe disease
          • Definitions

            • Langerhans cell histiocytosis (LCH): Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, S100 protein-positive dendritic cells
            • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
              • Bone (80-90%), skin (50%) most frequently involved
                • Lung most common SS site in adults
                • Skin only involvement in 2%
              • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis ("high risk")
                • Lung no longer considered RO
              • Other organs: Lymph nodes, pituitary, thymus, GI tract
              • CNS-risk lesion: Skull base & many facial lesions
                • At risk for diabetes insipidus & delayed neurodegenerative changes

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                          2. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                          3. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                          4. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                          5. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                          6. Atkin KL et al: The role of whole-body MRI in pediatric oncology. J Pediatr Hematol Oncol. 36(5):342-52, 2014
                                          7. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                          8. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                          9. Kamath S et al: Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution. J Pediatr Orthop. 34(8):825-30, 2014
                                          10. Lee JW et al: Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years' experience of 154 patients at a single center. Pediatr Hematol Oncol. 31(3):293-302, 2014
                                          11. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                          12. Jeh SK et al: Extracranial skeletal Langerhans cell histiocytosis: MR imaging features according to the radiologic evolutional phases. Clin Imaging. 36(5):466-71, 2012
                                          13. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                          14. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                          15. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                          16. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease, Letterer-Siwe disease
                                                  • Definitions

                                                    • Langerhans cell histiocytosis (LCH): Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, S100 protein-positive dendritic cells
                                                    • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
                                                      • Bone (80-90%), skin (50%) most frequently involved
                                                        • Lung most common SS site in adults
                                                        • Skin only involvement in 2%
                                                      • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis ("high risk")
                                                        • Lung no longer considered RO
                                                      • Other organs: Lymph nodes, pituitary, thymus, GI tract
                                                      • CNS-risk lesion: Skull base & many facial lesions
                                                        • At risk for diabetes insipidus & delayed neurodegenerative changes

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                                                                  2. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                                                                  3. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                                                                  4. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                                                                  5. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                                                                  6. Atkin KL et al: The role of whole-body MRI in pediatric oncology. J Pediatr Hematol Oncol. 36(5):342-52, 2014
                                                                                  7. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                                                                  8. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                                                                  9. Kamath S et al: Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution. J Pediatr Orthop. 34(8):825-30, 2014
                                                                                  10. Lee JW et al: Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years' experience of 154 patients at a single center. Pediatr Hematol Oncol. 31(3):293-302, 2014
                                                                                  11. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                                  12. Jeh SK et al: Extracranial skeletal Langerhans cell histiocytosis: MR imaging features according to the radiologic evolutional phases. Clin Imaging. 36(5):466-71, 2012
                                                                                  13. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                                                                  14. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                                                                  15. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                                                                  16. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003