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Sickle
cell anemia versus sickle cell diseases in adults
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Mehmet Rami Helvaci (1)
Hasan Yilmaz (2)
Atilla Yalcin (1)
Orhan Ekrem Muftuoglu (1)
Abdulrazak Abyad (3)
Lesley Pocock (4)
(1) Specialist of Internal Medicine,
MD
(2) Specialist of Ear, Nose, and Throat Diseases,
MD
(3) Middle-East Academy for Medicine of Aging,
MD
(4) medi-WORLD International
Corresponding author:
Prof Dr Mehmet Rami Helvaci,
07400, ALANYA, Turkey
Phone: 00-90-506-4708759
Email: mramihelvaci@hotmail.com
Received July 2021. Accepted
August 2021. Published September 1, 2021.Please
cite this article as: Helvaci MR et al. Acute
chest syndrome may not have an atherosclerotic
background in sickle cell diseases. Middle East
J Intern Med 2021; 14(2): 10- 19. DOI: 10.5742/MEJIM2020.93797.
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ABSTRACT
Background:
We tried to understand prevalence and clinical
severity of sickle cell anemia (SCA) alone or
sickle cell diseases (SCD) with associated alpha-
or beta-thalassemias in adults.
Methods: All adults with
the SCA or SCD were studied.
Results: The study included
441 patients (215 females). The prevalence of
SCA was significantly lower than the SCD in adults
(29.0% versus 70.9%, p<0.001). The mean age
and female ratio were similar in the SCA and SCD
groups (31.2 versus 30.5 years and 52.3% versus
47.2%, p>0.05 for both, respectively). The
mean body mass index was similar in both groups,
too (21.5 versus 21.7 kg/m2, p>0.05, respectively).
On the other hand, the total bilirubin value of
the plasma was higher in the SCA, significantly
(5.7 versus 4.4 mg/dL, p= 0.000). Whereas the
total number of transfused units of red blood
cells in their lives was similar in the SCA and
SCD groups (43.6 versus 37.1 units, p>0.05,
respectively).
Conclusion: The SCA alone
and SCD are severe inflammatory processes on vascular
endothelium particularly at the capillary level,
and terminate with an accelerated atherosclerosis
and end-organ failures in early years of life.
The relatively suppressed hemoglobin S synthesis
in the SCD secondary to the associated thalassemias
may decrease sickle cell-induced chronic endothelial
damage, inflammation, edema, fibrosis, and end-organ
failures. The lower prevalence of the SCA in adults
and the higher total bilirubin value of the plasma
in them may indicate the relative severity of
hemolytic process, vascular endothelial inflammation,
and hepatic involvement in the SCA.
Key words: Sickle cell
anemia, sickle cell diseases, thalassemias, chronic
endothelial damage, atherosclerosis, end-organ
failure, metabolic syndrome
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