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Histiocytic Disorders
Jorge Alberto Carrillo-Bayona, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          TERMINOLOGY

          • Abbreviations

            • Pulmonary Langerhans cell histiocytosis (PLCH)
              • Langerhans cell histiocytosis (LCH)
            • Erdheim-Chester disease (ECD)
            • Rosai-Dorfman disease (RDD)
          • Synonyms

            • RDD: Sinus histiocytosis with massive lymphadenopathy
          • Definitions

            • Rare disorders characterized by accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs
            • PLCH: Isolated form of LCH occurring in smokers characterized by granulomatous infiltration of distal bronchial walls with cells derived from myeloid dendritic cells (i.e., Langerhans cells)
            • ECD: Non-LCH dendritic cell disorder characterized by xanthomatous infiltration of involved tissues by foamy histiocytes
            • RDD: Nonmalignant proliferation of histiocytic &/or phagocytic cells

          IMAGING

          • Radiographic Findings

            • CT Findings

              • Nuclear Medicine Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Microscopic Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                Selected References

                                1. Vargas D et al: Cardiothoracic manifestations of primary histiocytoses. Br J Radiol. 89(1068):20160347, 2016
                                2. Ahuja J et al: Histiocytic disorders of the chest: imaging findings. Radiographics. 35(2):357-70, 2015
                                3. Antunes C et al: Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings. Insights Imaging. 5(4):473-82, 2014
                                4. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                5. Mazor RD et al: Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis. 8:137, 2013
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Pathology

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Pulmonary Langerhans cell histiocytosis (PLCH)
                                            • Langerhans cell histiocytosis (LCH)
                                          • Erdheim-Chester disease (ECD)
                                          • Rosai-Dorfman disease (RDD)
                                        • Synonyms

                                          • RDD: Sinus histiocytosis with massive lymphadenopathy
                                        • Definitions

                                          • Rare disorders characterized by accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs
                                          • PLCH: Isolated form of LCH occurring in smokers characterized by granulomatous infiltration of distal bronchial walls with cells derived from myeloid dendritic cells (i.e., Langerhans cells)
                                          • ECD: Non-LCH dendritic cell disorder characterized by xanthomatous infiltration of involved tissues by foamy histiocytes
                                          • RDD: Nonmalignant proliferation of histiocytic &/or phagocytic cells

                                        IMAGING

                                        • Radiographic Findings

                                          • CT Findings

                                            • Nuclear Medicine Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Microscopic Features

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Consider

                                                              Selected References

                                                              1. Vargas D et al: Cardiothoracic manifestations of primary histiocytoses. Br J Radiol. 89(1068):20160347, 2016
                                                              2. Ahuja J et al: Histiocytic disorders of the chest: imaging findings. Radiographics. 35(2):357-70, 2015
                                                              3. Antunes C et al: Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings. Insights Imaging. 5(4):473-82, 2014
                                                              4. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                              5. Mazor RD et al: Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis. 8:137, 2013