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Fat Embolism in Sickle Cell Disease: Report of a Case With Brief Review of the Literature

Fat Embolism in Sickle Cell Disease: Report of a Case With Brief Review of the Literature Abstract • This is a unique instance of fatal fat embolism secondary to bone marrow necrosis in a patient with sickle cell—hemoglobin C disease, systemic lupus erythematosus, and Sjögren's syndrome. An elevated level of IgG and treatment with corticosteroids appeared to be major factors in the death of this patient. This complication occurs most often in patients with sickle cell-hemoglobin C disease, especially in pregnant women near term. It should be suspected in any patient with sickle cell disease in whom unexplained fever, respiratory, and neurologic symptoms typical of fat emboli develop following the onset of bone pain. Exchange blood transfusion may be lifesaving. (Arch Intern Med 1984;144:181-182) References 1. Hutchinson RM, Merrick MV, White JM: Fat embolism in sickle cell disease. J Clin Pathol 1973;26:620-622.Crossref 2. Chmel H, Bertles JF: Hemoglobin S/C disease in a pregnant woman with crisis and fat embolization syndrome. Am J Med 1975;58:563-566.Crossref 3. Shelly WM, Curtis EM: Bone marrow and fat embolism in sickle cell anemia and sickle cell—hemoglobin C disease. Bull Johns Hopkins Hosp 1958;103:8-24. 4. Smith EW, Krevans JR: Clinical manifestations of hemoglobin C disorders. Bull Johns Hopkins Hosp 1959;27:647-658. 5. Rywlin AM, Block AL, Werner CS: Hemoglobin C and S disease in pregnancy. Am J Obstet Gynecol 1963;86:1055-1059. 6. Dines DE, Burgher LW, Okazaki H: The clinical and pathologic correlation of fat embolism syndrome. Mayo Clin Proc 1975;50:407-411. 7. Konotey-Ahulu FID: The sickle cell diseases: Clinical manifestations including the 'sickle crisis.' Arch Intern Med 1974;133:611-619.Crossref 8. Ober WB, Bruno MS, Simon RM, et al: Hemoglobin S-C disease with fat embolism. Am J Med 1959;27:647-658.Crossref 9. Diggs LW: Sickle cell crises. Am J Clin Pathol 1965;44:1-19. 10. Iffy L, Kaminetzsky HA: Principles and Practice of Obstetrics and Gynecology . New York, John Wiley & Sons Inc, 1981. 11. Reynolds J: A re-evaluation of the 'fish vertebra' sign in sickle cell hemoglobinopathy. AJR 1966;97:693-707.Crossref 12. Shearn MA, Epstein WV, Engleman EP: Serum viscosity in rheumatic diseases and macroglobulinemia. Arch Intern Med 1963;112:684-687. 13. Fukasawa T, Arai T, Naruse T, et al: Hyperviscosity syndrome in a patient with systemic lupus erythematosus. Am J Med Sci 1977;273:329-334.Crossref 14. Blaylock WM, Waller M, Normansell DE: Sjögren's syndrome: Hyperviscosity and intermediate complexes. Ann Intern Med 1974;80:27-34.Crossref 15. Pope RM, Fletcher MA, Mamby A, et al: Rheumatoid arthritis associated with hyperviscosity syndrome and intermediate complex formation. Arch Intern Med 1975;135:281-285.Crossref 16. Abeles M, Urman JD, Rothfield NF: Aseptic necrosis of bone in systemic lupus erythematosus: Relationship to corticosteroid therapy. Arch Intern Med 1978;138:750-754.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Fat Embolism in Sickle Cell Disease: Report of a Case With Brief Review of the Literature

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References (21)

Publisher
American Medical Association
Copyright
Copyright © 1984 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1984.00350130211039
Publisher site
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Abstract

Abstract • This is a unique instance of fatal fat embolism secondary to bone marrow necrosis in a patient with sickle cell—hemoglobin C disease, systemic lupus erythematosus, and Sjögren's syndrome. An elevated level of IgG and treatment with corticosteroids appeared to be major factors in the death of this patient. This complication occurs most often in patients with sickle cell-hemoglobin C disease, especially in pregnant women near term. It should be suspected in any patient with sickle cell disease in whom unexplained fever, respiratory, and neurologic symptoms typical of fat emboli develop following the onset of bone pain. Exchange blood transfusion may be lifesaving. (Arch Intern Med 1984;144:181-182) References 1. Hutchinson RM, Merrick MV, White JM: Fat embolism in sickle cell disease. J Clin Pathol 1973;26:620-622.Crossref 2. Chmel H, Bertles JF: Hemoglobin S/C disease in a pregnant woman with crisis and fat embolization syndrome. Am J Med 1975;58:563-566.Crossref 3. Shelly WM, Curtis EM: Bone marrow and fat embolism in sickle cell anemia and sickle cell—hemoglobin C disease. Bull Johns Hopkins Hosp 1958;103:8-24. 4. Smith EW, Krevans JR: Clinical manifestations of hemoglobin C disorders. Bull Johns Hopkins Hosp 1959;27:647-658. 5. Rywlin AM, Block AL, Werner CS: Hemoglobin C and S disease in pregnancy. Am J Obstet Gynecol 1963;86:1055-1059. 6. Dines DE, Burgher LW, Okazaki H: The clinical and pathologic correlation of fat embolism syndrome. Mayo Clin Proc 1975;50:407-411. 7. Konotey-Ahulu FID: The sickle cell diseases: Clinical manifestations including the 'sickle crisis.' Arch Intern Med 1974;133:611-619.Crossref 8. Ober WB, Bruno MS, Simon RM, et al: Hemoglobin S-C disease with fat embolism. Am J Med 1959;27:647-658.Crossref 9. Diggs LW: Sickle cell crises. Am J Clin Pathol 1965;44:1-19. 10. Iffy L, Kaminetzsky HA: Principles and Practice of Obstetrics and Gynecology . New York, John Wiley & Sons Inc, 1981. 11. Reynolds J: A re-evaluation of the 'fish vertebra' sign in sickle cell hemoglobinopathy. AJR 1966;97:693-707.Crossref 12. Shearn MA, Epstein WV, Engleman EP: Serum viscosity in rheumatic diseases and macroglobulinemia. Arch Intern Med 1963;112:684-687. 13. Fukasawa T, Arai T, Naruse T, et al: Hyperviscosity syndrome in a patient with systemic lupus erythematosus. Am J Med Sci 1977;273:329-334.Crossref 14. Blaylock WM, Waller M, Normansell DE: Sjögren's syndrome: Hyperviscosity and intermediate complexes. Ann Intern Med 1974;80:27-34.Crossref 15. Pope RM, Fletcher MA, Mamby A, et al: Rheumatoid arthritis associated with hyperviscosity syndrome and intermediate complex formation. Arch Intern Med 1975;135:281-285.Crossref 16. Abeles M, Urman JD, Rothfield NF: Aseptic necrosis of bone in systemic lupus erythematosus: Relationship to corticosteroid therapy. Arch Intern Med 1978;138:750-754.Crossref

Journal

Archives of Internal Medicine – American Medical Association

Published: Jan 1, 1984

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