|
Abstract
Background: Sickle
cell diseases (SCDs) are inborn
and destructive processes
on vascular endothelium, particularly
at the capillaries.
Methods:
Patients with red blood cells
(RBCs) transfusions of less
than 50 units in their lives
were put into the first and
50 units or higher were put
into the second groups.
Results:
There were 224 patients in
the first and 92 patients
in the second groups. Mean
ages were similar in them
(28.9 vs 30.0 years, respectively,
p>0.05). The male ratio
was higher in the second group
(45.5% vs 64.1%, p<0.001).
In contrast to the lower prevalence
of autosplenectomy (56.2%
vs 45.6%, p<0.05), painful
crises per year, digital clubbing,
chronic obstructive pulmonary
disease (COPD), leg ulcers,
stroke, chronic renal disease
(CRD), and coronary heart
disease (CHD) were all higher
in the second group (p<0.05
for all).
Conclusion:
The sickled or just hardened
RBCs-induced capillary endothelial
damage initiates at birth,
and terminates with multiorgan
failures even at childhood.
Although RBCs suspensions
and corticosteroids in acute,
and aspirin with an anti-inflammatory
dose plus low-dose warfarin
plus hydroxyurea both in acute
and chronic phases decrease
severity, survivals are still
shortened in both genders,
dramatically. In contrast
to the lower prevalence of
autosplenectomy, painful crises
per year, digital clubbing,
COPD, leg ulcers, stroke,
CRD, and CHD were higher in
the second group. So there
may be an inverse relationship
between prevalence of autosplenectomy
and severity of SCDs, and
spleen may act as a chronic
inflammatory focus as a filter
of blood for these abnormally
hardened RBCs.
Key
words: Sickle cell diseases,
sickled or just hardened red
blood cells, capillary endothelial
edema, myocardial infarction,
stroke, sudden deaths, autosplenectomy
|
