Correspondence

Pulmonary Complications of Sickle Cell Disease

N Engl J Med 2009; 360:1044-1045March 5, 2009

Article

To the Editor:

In their review, Gladwin and Vichinsky (Nov. 20 issue)1 list events that can precipitate acute pulmonary complications of sickle cell disease, but the inflammatory response after surgical procedures was not discussed. The very high incidence of postoperative complications in sickle cell disease has been assumed to be due to increased sickling.2 However, limited data from humans exposed to severe acute and chronic hypoxia suggest that increased sickling does not invariably trigger vaso-occlusion.3,4 No dose effect of the dilution or removal of sickle erythrocytes by means of aggressive transfusion was detected in one large study.2 In the postoperative setting, chronic vascular inflammation may play a more prominent role than sickling.3,4 Management with hyperoxygenation, aggressive hydration, and blood transfusion should therefore be reexamined.

Paul G. Firth, M.B., Ch.B.
Massachusetts General Hospital, Boston, MA 02114
pfirth@partners.org

4 References

1. 1

   Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med 2008;359:2254-2265
   Full Text | Web of Science | Medline

2. 2

   Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med 1995;333:206-213
   Full Text | Web of Science | Medline

3. 3

   Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology 2004;101:766-785
   CrossRef | Web of Science | Medline

4. 4

   Firth PG. Anaesthesia for peculiar cells -- a century of sickle cell disease. Br J Anaesth 2005;95:287-299
   CrossRef | Web of Science | Medline

Author/Editor Response

In response to Firth: we agree with the need for prospective, randomized trials involving patients with sickle cell disease who have undergone surgery. The Preoperative Transfusion Sickle Cell Study Group is the only prospective, randomized trial of a standardized perioperative protocol in sickle cell disease. The 1127 enrollees included 179 patients who had not received a transfusion and were followed in a registry. Since cholecystectomy was the most common surgery, it was used to compare the effects of transfusion with no transfusion on complication rates.1 A total of 38% of the group of patients who had not received a transfusion had an acute sickle cell complication as compared with 12% of the aggressive-transfusion group and 18% of the conservative-transfusion group. Acute chest syndrome was twice as common in the patients who had not received a transfusion. The mortality in this group was 5% as compared with 0% in the transfusion groups. Our more recent prospective trials using preoperative transfusion protocols continue to provide support for its further use.2 At present, the data support preoperative transfusion in patients with sickle cell disease who are undergoing major surgery, despite the limited power in one trial to detect a dose effect.

Mark T. Gladwin, M.D.
University of Pittsburgh, Pittsburgh, PA 15213
gladwinmt@upmc.edu

Gregory J. Kato, M.D.
National Institutes of Health, Bethesda, MD 20892

Elliott Vichinsky, M.D.
Children's Hospital and Research Center at Oakland, Oakland, CA 94609

2 References

1. 1

   Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Blood 1997;89:1533-1542
   Web of Science | Medline

2. 2

   Neumayr LD, Aguilar C, Earles AN, et al. Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease: results of a multicenter study at a mean of three years after treatment. J Bone Joint Surg Am 2006;88:2573-2582
   CrossRef | Web of Science | Medline

Citing Articles (2)

Citing Articles

1. 1

   Paul G. Firth, Kristen N. McMillan, Charles M. Haberkern, Myron Yaster, Michael A. Bender, Salvatore R. Goodwin. (2011) A survey of perioperative management of sickle cell disease in North America. Pediatric Anesthesia 21:1, 43-49
   CrossRef

2. 2

   Paul G. Firth. (2009) Anesthesia and Hemoglobinopathies. Anesthesiology Clinics 27:2, 321-336
   CrossRef