link
Bookmarks
Chest
Diagnosis
Cardiovascular Disorders
Pulmonary Hypertension and Thromboembolic Disease
Sickle Cell Disease
Sickle Cell Disease
Aletta Ann Frazier, MD
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
13
12
1

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
                                        Loading...
                                        Related Differential Diagnoses
                                        Loading...
                                        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