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Fatal Pulmonary Artery Embolism in a Sickle Cell Patient: Case Report and Literature Review

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Abstract

This article reports a rare case of fatal saddle embolism to the pulmonary artery presenting as an Acute Chest Syndrome (ACS) in a Sickle Cell patient. We present a review of the etiology, pathophysiology, clinical manifestation and management of ACS. Clinicians should be aware of the fact that a sickle cell crisis admission may represent a life-threatening process. Such awareness will help physicians to act promptly and execute proper therapeutic interventions. It is important for clinicians to be expectant of impending clinical deterioration and likewise be aware that ACS can develop in patients hospitalized for other medical or surgical conditions.

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References

  1. Quinn CT, Buchanan GR. The acute chest syndrome of sickle cell disease. J Pediatr1999;135: 416–422.

    Google Scholar 

  2. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the Acute Chest Syndrome in sickle cell disease. NEJM2000;342: 1855–1865.

    Google Scholar 

  3. Powers D, Weidman JA, Odom-Maryon T, Niland JC, Johnson C. Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine1988;67: 66–76.

    Google Scholar 

  4. Van Agtmael MA, Cheng JD, Nossent HC. Acute chest syndrome in adult Afro-Caribbean patients with sickle cell disease. Arch Intern Med1994;154: 557–561.

    Google Scholar 

  5. Sprinkle RH, Cole T, Smith S, Buchanan GR. Acute chest syndrome in children with sickle cell disease. Am J Pediatr. Hematol Oncol 1986;8: 105–110.

    Google Scholar 

  6. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr1985;107: 861–866.

    Google Scholar 

  7. Davies SC, Luce PJ, Win AA, Riordan JF. Acute chest syndrome in sickle-cell disease. Lancet1984;1: 36–38.

    Google Scholar 

  8. Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood1997;89: 1787–1792.

    Google Scholar 

  9. Bernini JC, Quinn CT, Sandler ES, Reisch JA, Quinn CT, Buchanan GR. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood1998;92: 3082–3089.

    Google Scholar 

  10. Poncz M, Greenberg J, Gill FM, Cohen A. Hematologic changes during acute chest syndrome in sickle cell anemia. Am J Pediatr Hematol Oncol1985;7: 96–99.

    Google Scholar 

  11. Haynes J Jr, Michael BK. The acute chest syndrome of sickle cell disease. Am J Med Sci1993;305: 326–330.

    Google Scholar 

  12. Dreyer ZE. Chest infections and syndromes in sickle cell disease of childhood. Semin Respir Infect1996;11: 163–172.

    Google Scholar 

  13. Hassell KL, Eckman JR, Lane PA. Acute multi-organ failure syndrome: a potentially catastrophic complication of severe sickle cell pain episodes. Am J Med1994;96: 155–162.

    Google Scholar 

  14. Vichinsky E, Williams R, Das M, et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood1994;83: 3107–3112.

    Google Scholar 

  15. Van de Pette JEW, Pearson TC, Slater NGP. Exchange transfusion in life threatening sickling crises. J R Soc Med1982;75: 777–780.

    Google Scholar 

  16. Barrett-Connor E. Acute pulmonary disease in sickle cell anemia. Am Rev Respir Kis1971;104: 159–165.

    Google Scholar 

  17. Barrett-Connor E. Pneumonia and pulmonary infarction in sickle cell anemia. JAMA1973;224: 997–1000.

    Google Scholar 

  18. Oppenheimer EH, Esterly JR. Pulmonary changes in sickle cell disease. Am Rev Respir Dis1971;103: 858–859.

    Google Scholar 

  19. Steinberg B. Sickle cell anemia. Arch Pathol1930;9: 876–897.

    Google Scholar 

  20. Haupt HM, Moore W, Bauer TW, Hutchins GM. The lung in sickle cell disease. Chest1982;81: 332–337.

    Google Scholar 

  21. Bhalla M, Abboud MR, McCloud TC, et al. Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology1993;187: 45–49.

    Google Scholar 

  22. Walker BK, Ballas SK, Burka ER. The diagnosis of pulmonary thromboembolism in sickle cell disease. Am J Hematol1979;7: 219–232.

    Google Scholar 

  23. Babiker MA, Obeid HA, Ashong EF. Acute reversible pulmonary ischemia: a cause of the acute chest syndrome in sickle cell disease. Clin Pediatr1985;24: 716–718.

    Google Scholar 

  24. Aldrich TK, Dhuper SK, Patwa NS, et al. Pulmonary entrapment of sickle cells: the role of regional alveolar hypoxia. J Appl Physiol1996;80: 531–539.

    Google Scholar 

  25. Emre U, Miller ST, Gutierez M, Steiner P, Rao SP, Rao M. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr1995;127: 901–904.

    Google Scholar 

  26. Mallouh AA, Asha M. Beneficial effect of blood transfusion in children with sickle cell chest syndrome. Am J Dis Child1988;142: 178–182.

    Google Scholar 

  27. Warrier RP, Ducos R, Yu LC. Acute chest syndrome in sickle cell disease (letter). J Pediatr1986;109: 731.

    Google Scholar 

  28. Lanzkowsky P, Shende A, Karayalcin G, Kim Y-J, Aballi AJ. Partial exchange transfusion in sickle cell anemia: use in children with serious complications. Am J Dis Child1978;132: 1206–1208.

    Google Scholar 

  29. Bodo I, Khoury H, Blinder M. Rapid resolution of the acute chest syndrome of sickle cell disease after automated red cell exchange (abstract). Blood1997;90(suppl 1):23b.

    Google Scholar 

  30. Levy D. The fat embolism syndrome: a review. Clin Orthop1990;261(2): 81–86.

    Google Scholar 

  31. Moylan JA, Birnbaum M, Katz A, Everson MA. Fat emboli syndrome. J Trauma1976;16: 341–347.

    Google Scholar 

  32. Schuster DP. ARDS: clinical lessons from the oleic acid model of acute lung injury. Am J Respir Crit Care Med1994;149: 245–260.

    Google Scholar 

  33. Styles LA, Schalkwijk CG, Aarsman AJ, Vichinsky EP, Lubin BH, Kuypers FA. Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood1996;87: 2573–2578.

    Google Scholar 

  34. Moser KM, Shea JG. The relationship between pulmonary infarction, cor pulmonale and the sickle states. Am J Med1957;22: 561–579.

    Google Scholar 

  35. Diggs LW, Barreras L. Pulmonary emboli vs. pneumonia in patients with sickle cell anemia. Memphis Mid-South Med J1967;42: 375–380.

    Google Scholar 

  36. Ballas SK, Park CH. Severe hypoxemia secondary to acute sternal infarction in sickle cell anemia. J Nucl Med1991;32: 1617–1678.

    Google Scholar 

  37. Palmer J, Broderick KA, Naiman JL. Acute lung syndrome during painful sickle cell crisis: relation to site of pain and narcotic requirement (abstract). Blood1983;62: 59a.

    Google Scholar 

  38. Maitre B, Habibia. Acute chest syndrome in adults with sickle cell disease. Chest2000;117.

  39. Atz AM, Wessel DL. Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology1997;87: 988–990.

    Google Scholar 

  40. Walker BK, Ballas SK. The diagnosis of pulmonary thromboembolism in sickle cell disease. American Journal of Hematology1979;7: 219–232.

    Google Scholar 

  41. Robin ED. Overdiagnosis and overtreatment of pulmonary embolism: the emperor may have no clothes. Ann Intern Med1997;87: 775–781.

    Google Scholar 

  42. McIntyre KM. Pulmonary thromboembolism: current concepts. Adv Intern Med1972;18: 199–218.

    Google Scholar 

  43. Miller GJ. An assessment of lung volumes and gas transfer in sickle cell anemia. Thorax1971;26: 309–315.

    Google Scholar 

  44. Sproule B. A study of cardiopulmonary alterations in patients with sickle cell disease and its variants. J Clin Invest1958;37: 486–495.

    Google Scholar 

  45. Richards D. Angiographic media and the sickling phenomenon. Surg Forum1971;22: 403–404.

    Google Scholar 

  46. Cheatham ML. Problems in the management of subarachnoid hemorrhage in sickle cell anemia. J Neurosurg1965;23: 488–493.

    Google Scholar 

  47. Petch MC. Clinical features of pulmonary lesions in sickle cell anemia. Br Med J1970;3: 31.

    Google Scholar 

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Authors and Affiliations

  1. Department of Cardiology, Creighton University School of Medicine, Omaha, NE, 68131, USA

    Dhanunjaya R. Lakkireddy

  2. Department of Internal Medicine, UMKC School of Medicine, Kansas City, MO, 64131, USA

    Robert Patel

  3. Department of Internal Medicine, Sinai Grace Hospital, Wayne State University, Detroit, MI, 48201, USA

    Krishnamohan Basarakodu

  4. Department of Cardiovascular Sciences, KU Medical Center, University of Kansas, Kansas City, KS, 66160, USA

    James Vacek

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  1. Dhanunjaya R. Lakkireddy
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  2. Robert Patel
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  3. Krishnamohan Basarakodu
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  4. James Vacek
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Lakkireddy, D.R., Patel, R., Basarakodu, K. et al. Fatal Pulmonary Artery Embolism in a Sickle Cell Patient: Case Report and Literature Review. J Thromb Thrombolysis 14, 79–83 (2002). https://doi.org/10.1023/A:1022074507216

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  • DOI: https://doi.org/10.1023/A:1022074507216

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