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Chest
Diagnosis
Cardiovascular Disorders
Pulmonary Hypertension and Thromboembolic Disease
Sickle Cell Disease
Sickle Cell Disease
Aletta Ann Frazier, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Sickle cell disease (HbSS)
                • Acute chest syndrome (ACS)

              • Definitions

                • HbSS: Inherited hemoglobinopathy; abnormal hemoglobin molecule deforms when deoxygenated
                • Spectrum of inherited blood disorders
                • Acute chest syndrome: Respiratory symptoms ± fever in HbSS patients and new chest radiographic abnormalities
                  • Often preceded by vaso-occlusive crisis with bone pain
                    • ~ 80% adults with ACS
                  • May be precipitated by lung infection ± infarction
                  • Adult patients face greater severity and risk of death
                • Sickle cell chronic lung disease: Result of recurrent ACS
                • Sickle cell trait: Strictly not HbSS; benign clinical course

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Ogu UO et al: Management of sickle cell disease complications beyond acute chest syndrome. J Blood Med. 12:101-14, 2021
                                        2. Carden MA et al: Emerging disease-modifying therapies for sickle cell disease. Haematologica. 104(9):1710-9, 2019
                                        3. Carstens GR et al: Clinical relevance of pulmonary vasculature involvement in sickle cell disease. Br J Haematol. 185(2):317-26, 2019
                                        4. Neumayr LD et al: Sickle cell disease: current treatment and emerging therapies. Am J Manag Care. 25(18 Suppl):S335-43, 2019
                                        5. Savale L et al: Clinical phenotypes and outcomes of precapillary pulmonary hypertension of sickle cell disease. Eur Respir J. 54(6), 2019
                                        6. Sundd P et al: Pathophysiology of sickle cell disease. Annu Rev Pathol. 14:263-92, 2019
                                        7. Swift CA et al: Incidence of pulmonary embolism in sickle cell anemia patients undergoing computed tomography pulmonary angiography in the emergency department. J Thorac Imaging. 34(5):W125-6, 2019
                                        8. Kato GJ et al: Sickle cell disease. Nat Rev Dis Primers. 4:18010, 2018
                                        9. Kato GJ et al: Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest. 127(3):750-60, 2017
                                        10. Ware RE et al: Sickle cell disease. Lancet. 390(10091):311-23, 2017
                                        11. Mehari A et al: Chronic pulmonary complications of sickle cell disease. Chest. 149(5):1313-24, 2016
                                        12. Klings ES et al: An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 189(6):727-40, 2014
                                        13. Hasleton P et al: Pulmonary vascular pathology. In Spencer's Pathology of the Lung. 6th ed. University of Cambridge Press. 679-80, 2013
                                        14. Miller AC et al: Pulmonary complications of sickle cell disease. Am J Respir Crit Care Med. 185(11):1154-65, 2012
                                        15. Voskaridou E et al: Sickle-cell disease and the heart: review of the current literature. Br J Haematol. 157(6):664-73, 2012
                                        16. Gladwin MT et al: Pulmonary complications of sickle cell disease. N Engl J Med. 359(21):2254-65, 2008
                                        17. Delclaux C et al: Factors associated with dyspnea in adult patients with sickle cell disease. Chest. 128(5):3336-44, 2005
                                        18. Siddiqui AK, Ahmed S. Pulmonary manifestations of sickle cell disease. Postgrad Med J. 79(933):384-90, 2003
                                        19. Maitre B et al: Acute chest syndrome in adults with sickle cell disease. Chest. 117(5):1386-92, 2000
                                        20. Vichinsky EP et al: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 342(25):1855-65, 2000
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Sickle cell disease (HbSS)
                                                      • Acute chest syndrome (ACS)

                                                    • Definitions

                                                      • HbSS: Inherited hemoglobinopathy; abnormal hemoglobin molecule deforms when deoxygenated
                                                      • Spectrum of inherited blood disorders
                                                      • Acute chest syndrome: Respiratory symptoms ± fever in HbSS patients and new chest radiographic abnormalities
                                                        • Often preceded by vaso-occlusive crisis with bone pain
                                                          • ~ 80% adults with ACS
                                                        • May be precipitated by lung infection ± infarction
                                                        • Adult patients face greater severity and risk of death
                                                      • Sickle cell chronic lung disease: Result of recurrent ACS
                                                      • Sickle cell trait: Strictly not HbSS; benign clinical course

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Nuclear Medicine Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Ogu UO et al: Management of sickle cell disease complications beyond acute chest syndrome. J Blood Med. 12:101-14, 2021
                                                                              2. Carden MA et al: Emerging disease-modifying therapies for sickle cell disease. Haematologica. 104(9):1710-9, 2019
                                                                              3. Carstens GR et al: Clinical relevance of pulmonary vasculature involvement in sickle cell disease. Br J Haematol. 185(2):317-26, 2019
                                                                              4. Neumayr LD et al: Sickle cell disease: current treatment and emerging therapies. Am J Manag Care. 25(18 Suppl):S335-43, 2019
                                                                              5. Savale L et al: Clinical phenotypes and outcomes of precapillary pulmonary hypertension of sickle cell disease. Eur Respir J. 54(6), 2019
                                                                              6. Sundd P et al: Pathophysiology of sickle cell disease. Annu Rev Pathol. 14:263-92, 2019
                                                                              7. Swift CA et al: Incidence of pulmonary embolism in sickle cell anemia patients undergoing computed tomography pulmonary angiography in the emergency department. J Thorac Imaging. 34(5):W125-6, 2019
                                                                              8. Kato GJ et al: Sickle cell disease. Nat Rev Dis Primers. 4:18010, 2018
                                                                              9. Kato GJ et al: Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest. 127(3):750-60, 2017
                                                                              10. Ware RE et al: Sickle cell disease. Lancet. 390(10091):311-23, 2017
                                                                              11. Mehari A et al: Chronic pulmonary complications of sickle cell disease. Chest. 149(5):1313-24, 2016
                                                                              12. Klings ES et al: An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 189(6):727-40, 2014
                                                                              13. Hasleton P et al: Pulmonary vascular pathology. In Spencer's Pathology of the Lung. 6th ed. University of Cambridge Press. 679-80, 2013
                                                                              14. Miller AC et al: Pulmonary complications of sickle cell disease. Am J Respir Crit Care Med. 185(11):1154-65, 2012
                                                                              15. Voskaridou E et al: Sickle-cell disease and the heart: review of the current literature. Br J Haematol. 157(6):664-73, 2012
                                                                              16. Gladwin MT et al: Pulmonary complications of sickle cell disease. N Engl J Med. 359(21):2254-65, 2008
                                                                              17. Delclaux C et al: Factors associated with dyspnea in adult patients with sickle cell disease. Chest. 128(5):3336-44, 2005
                                                                              18. Siddiqui AK, Ahmed S. Pulmonary manifestations of sickle cell disease. Postgrad Med J. 79(933):384-90, 2003
                                                                              19. Maitre B et al: Acute chest syndrome in adults with sickle cell disease. Chest. 117(5):1386-92, 2000
                                                                              20. Vichinsky EP et al: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 342(25):1855-65, 2000