Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death

doi:10.1056/NEJM199406093302303

Volume 330:1639-1644		June 9, 1994		Number 23

Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death

Orah S. Platt, Donald J. Brambilla, Wendell F. Rosse, Paul F. Milner, Oswaldo Castro, Martin H. Steinberg, and Panpit P. Klug

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ABSTRACT

Background Information on life expectancy and risk factors forearly death among patients with sickle cell disease (sicklecell anemia, sickle cell-hemoglobin C disease, and the sicklecell- ${beta}$ -thalassemias) is needed to counsel patients, target therapy,and design clinical trials.

Methods We followed 3764 patients who ranged from birth to 66years of age at enrollment to determine the life expectancyand calculate the median age at death. In addition, we investigatedthe circumstances of death for all 209 adult patients who diedduring the study, and used proportional-hazards regression analysisto identify risk factors for early death among 964 adults withsickle cell anemia who were followed for at least two years.

Results Among children and adults with sickle cell anemia (homozygousfor sickle hemoglobin), the median age at death was 42 yearsfor males and 48 years for females. Among those with sicklecell-hemoglobin C disease, the median age at death was 60 yearsfor males and 68 years for females. Among adults with sicklecell disease, 18 percent of the deaths occurred in patientswith overt organ failure, predominantly renal. Thirty-threepercent were clinically free of organ failure but died duringan acute sickle crisis (78 percent had pain, the chest syndrome,or both; 22 percent had stroke). Modeling revealed that in patientswith sickle cell anemia, the acute chest syndrome, renal failure,seizures, a base-line white-cell count above 15,000 cells percubic millimeter, and a low level of fetal hemoglobin were associatedwith an increased risk of early death.

Conclusions Fifty percent of patients with sickle cell anemiasurvived beyond the fifth decade. A large proportion of thosewho died had no overt chronic organ failure but died duringan acute episode of pain, chest syndrome, or stroke. Early mortalitywas highest among patients whose disease was symptomatic. Ahigh level of fetal hemoglobin predicted improved survival andis probably a reliable childhood forecaster of adult life expectancy.

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From the Department of Medicine, Children's Hospital, the Dana-Farber Cancer Institute, and Harvard Medical School -- all in Boston (O.S.P.); New England Research Institute, Watertown, Mass. (D.J.B.); the Department of Medicine, Duke University School of Medicine, Durham, N.C. (W.F.R.); the Department of Medicine, Medical College of Georgia, Augusta (P.F.M.); the Department of Medicine, Center for Sickle Cell Disease, Howard University, Washington, D.C. (O.C.); the Hematology-Oncology Section, Jackson Veterans Affairs Medical Center, and the Department of Medicine, University of Mississippi School of Medicine -- both in Jackson (M.H.S.); and the Hematology Division, Texas Tech University Health Science Center, Amarillo (P.P.K.). The investigators who participated in this study are listed in the Appendix.

Address reprint requests to Dr. Platt at the Department of Laboratory Medicine, Children's Hospital, 300 Longwood Ave., Boston, MA 02115.

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Mortality in Sickle Cell Disease
Rodgers G. P., Walker E. C., Podgor M. J., Platt O. S.
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N Engl J Med 1994; 331:1022-1023, Oct 13, 1994. Correspondence

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