Annals of Hematology

, Volume 95, Issue 6, pp 993–1000 | Cite as

East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients

  • Pelin Kardaş Karacaoglu
  • Suheyl Asma
  • Aslı Korur
  • Soner Solmaz
  • Nurhilal Turgut Buyukkurt
  • Cigdem Gereklioglu
  • Mutlu Kasar
  • Demircan Ozbalcı
  • Selma Unal
  • Hasan Kaya
  • Emel Gurkan
  • Mahmut Yeral
  • Çagla Sariturk
  • Can Boga
  • Hakan Ozdogu
Original Article

Abstract

Sickle cell disease (SCD), one of the most common genetic disorders worldwide, is characterized by hemolytic anemia and tissue damage from the rigid red blood cells. Although hydroxyurea and transfusion therapy are administered to treat the accompanying tissue injury, whether either one prolongs the lifespan of patients with SCD is unknown. SCD-related mortality data are available, but there are few studies on mortality-related factors based on evaluations of surviving patients. In addition, ethnic variability in patient registries has complicated detailed analyses. The aim of this study was to investigate mortality and mortality-related factors among an ethnically homogeneous population of patients with SCD. The 735 patients (102 children and 633 adults) included in this retrospective cohort study were of Eti-Turk origin and selected from 1367 patients seen at 5 regional hospitals. A central population management system was used to control for records of patient mortality. Data reliability was checked by a data supervision group. Mortality-related factors and predictors were identified in univariate and multivariate analyses using a Cox regression model with stepwise forward selection. The study group included patients with homozygous hemoglobin S (Hgb S) disease (67 %), Hb S-β0 thalassemia (17 %), Hgb S-β+ thalassemia (15 %), and Hb S-α thalassemia (1 %). They were followed for a median of 66 ± 44 (3–148) months. Overall mortality at 5 years was 6.1 %. Of the 45 patients who died, 44 (6 %) were adults and 1 (0.1 %) was a child. The mean age at death was 34.1 ± 10 (18–54) years for males, 40.1 ± 15 (17–64) years for females, and 36.6 ± 13 (17–64) years overall. Hydroxyurea was found to have a notable positive effect on mortality (p = 0.009). Mortality was also significantly related to hypertension and renal damage in a univariate analysis (p = 0.015 and p = 0.000, respectively). Acute chest syndrome, splenic sequestration, and prolonged painful-crisis-related multiorgan failure were the most common causes of mortality. In a multivariate analysis of laboratory values, only an elevated white blood cell count was related to mortality (p = 0.009). These data show that despite recent progress in the treatment of SCD, disease-related factors continue to result in mortality in young adult patients. Our results highlight the importance of evaluating curative treatment options for patients who have an appropriate stem cell donor in addition to improving patient care and patient education.

Keywords

Sickle cell disease Mortality Painful crisis Acute chest syndrome Hemoglobinopathy 

Notes

Compliance with ethical standard

This study was approved by the Institutional Medical and Health Sciences Experimental/Clinical Research Principles and Research Committee (project number: KA15/07). The required approvals for the collaborations in the study were obtained from the concerned departments and hospitals.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Pelin Kardaş Karacaoglu
    • 1
  • Suheyl Asma
    • 2
  • Aslı Korur
    • 2
  • Soner Solmaz
    • 3
  • Nurhilal Turgut Buyukkurt
    • 3
  • Cigdem Gereklioglu
    • 2
  • Mutlu Kasar
    • 3
  • Demircan Ozbalcı
    • 4
  • Selma Unal
    • 5
  • Hasan Kaya
    • 6
  • Emel Gurkan
    • 7
  • Mahmut Yeral
    • 3
  • Çagla Sariturk
    • 8
  • Can Boga
    • 3
  • Hakan Ozdogu
    • 3
  1. 1.Department of Internal MedicineBaskent University Faculty of MedicineAnkaraTurkey
  2. 2.Department of Internal Medicine, Family Medicine DivisionBaskent University Faculty of MedicineAnkaraTurkey
  3. 3.Department of Internal Medicine, Hematology DivisionBaskent University Faculty of MedicineAnkaraTurkey
  4. 4.Mersin State Hospital, Hematology DivisionMersinTurkey
  5. 5.Department of Internal Medicine, Pediatric Hematology DivisionMersin University Faculty of MedicineMersinTurkey
  6. 6.Department of Internal Medicine, Hematology DivisionMustafa Kemal University Faculty of MedicineAntakyaTurkey
  7. 7.Department of Internal Medicine, Hematology DivisionCukurova University Faculty of MedicineAdanaTurkey
  8. 8.Medical statisticsBaskent University Faculty of MedicineAdanaTurkey

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