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Estimation of serum ferritin level of a cohort of patients with sickle cell anemia in Port Harcourt, Nigeria.

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ISSN No. 2394-3971

Original Research Article

ESTIMATION OF SERUM FERRITIN LEVEL OF A COHORT OF PATIENTS WITH
SICKLE CELL ANEMIA IN PORT HARCOURT, NIGERIA.
D.A.Okoh1, C.O. Nwabuko2,3

1. Department of Pathology, Braithwaite Memorial Specialist Hospital, Port Harcourt, Rivers

State, Nigeria.
2. Division of Haematology, Department of Internal Medicine, Abia State University, Aba, Abia
State, Nigeria.
3. Department of Haematology and Blood Transfusion, Federal Medical Center, Umuahia, Abia
State, Nigeria.

Abstract
Background: Iron overload remains a significant clinical concern in sickle cell anemia (SCA)
despite contrary reports of adequate iron stores and overt iron deficiency anemia in SCA. It has
therefore, become imperative to evaluate the iron status of sickle cell patients in Rivers state
Nigeria.
Methodology: This was a Prospective Study of 108 subjects comprising of 54 SCA patients and
54 aged, sex matched normal (HbAA) control. Serum ferritin was determined by
spectrophotometric method.
Result: The SCA patients had a mean serum ferritin level of 300200 g/L (range; 80 to 1,200
g/L) which was higher than the mean of 8822 g/L (range 12-150g) of the control group (P
< 0.001). There was a weak direct correlation between serum ferritin concentration and the
number of units of blood transfused (r = 0.25 (P = 0.061). Forty three (80%) of the SCA patients
had increased iron stores while eleven (20%) had adequate iron stores comparable to the healthy
control group. There was no serum ferritin level suggestive of iron deficiency anemia from this
study.
Conclusion: We therefore recommend sequential iron studies for patients on recurrent blood
transfusion, while iron supplementation should only be given in established cases of iron
deficiency anemia.
Keywords: SCA, Serum Ferritin, Iron status.
Introduction
Sickle cell disease (SCD) refers to a group of
disorders caused by the co-dominant
inheritance of two abnormal hemoglobin
genes including the sickle hemoglobin (HbS)
gene. These disorders include the
homozygote sickle cell anemia (HbSS) and

compound heterozygotes such as HbSC, SOArab, Sthal and SD.1 The mutant
hemoglobin S molecule (HbS) is the
dominant factor in the cascade of cellular
events, responsible for the pathogenesis of
the clinical features associated with SCD.
These include chronic hemolytic anemia,

Okoh D. A. & Nwabuko C. O., Med. Res. Chron., 2015, 3 (1), 97-101

Medico Research Chronicles, 2016

Submitted on: February 2016

Accepted on: February 2016
For Correspondence
Email ID:

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vaso-occlusive events (painful crisis),

multiple organ damage and chronic
vasculopathy triggered by free heme1, 2. The
homozygous form (HbSS) or sickle cell
anemia however, remains the most clinically
severe phenotype with high mortality.
Sickle cell anemia (SCA) is often
characterized by severe anemia and recurrent
vaso-occlusive complications which often are
managed with various blood transfusion
regimens.3,4 Consequently, SCA patients are
potentially at risk of iron overload from
chronic blood transfusion, in addition to
increased intestinal absorption of iron. Iron
overload causes progressive tissue toxicity,
leading to cell death, fibrosis, and organ
dysfunction which could be fatal without
chelation therapy5-7. While iron overload
remains a clinical concern in SCA, various
studies have also shown that Iron deficiency
may not be uncommon, especially in the
tropics8-10. This is attributed to poverty,
nutritional deficiencies, and parasitic
infections. Iron deficiency in sickle cell
anemia is known to precipitate hemolytic and
vaso- occlusive crises as well as impair the
growth and intellectual performance of
affected individuals.8
There is currently a paucity of information on
the iron status of SCA patients in the Niger
Delta region. Hence, it has become
imperative to evaluate the serum ferritin
levels of SCA patients in Rivers state. This is
aimed at providing optimum medical care for
patients with sickle cell anemia. Serum
ferritin concentration which mainly reflects
reticuloendothelial iron store is often used as
a surrogate marker for body iron status.
Ferritin is also an acute phase reactant which
may be raised in chronic inflammation,
infections and liver disorders independent of
iron status. However, none of the subjects
recruited into this study had any chronic
illness and all the SCA patients were in
steady state 11.

Materials and Methods

A total of 54 consecutive SCA patients aged
(4 - 27 years) and 54 age and sex-matched
healthy controls (HbAA) participated in the
study between November 1999 and August
2000 at the Haematology SCD clinic of the
University of Port Harcourt Teaching
Hospital, Rivers State- Nigeria.
Ethical approval for the study was obtained
from the hospital ethical committee before
the commencement of the study, while
written informed consent was obtained from
the adult subjects, and parents/guardians of
younger participants before recruitment.
Those who fulfilled the study criteria were
recruited. The inclusion criteria included
consenting SCD subjects (confirmed by
hemoglobin electrophoresis) in steady state
(absence of any crisis in the preceding four
weeks and absence of any symptom or sign
attributable to acute illness). Some of the
criteria used were also applicable to healthy
control who were phenotypically (HbAA)
confirmed individuals. It was also ensured
that none of the control subjects received any
iron supplementation within three months
prior to recruitment.
The exclusion criteria however included:
refusal of consent, SCA subjects in crises or
suffering from any chronic illness not related
to sickle cell anemia, all subjects who had
blood transfusion therapy within three
months prior to study and all females who
were menstruating or pregnant.
The sampling procedure was explained to the
participants and about 5mls of venous blood
was aseptically collected by venepuncture
into plain tubes using a wide 20 gauge
needle. The samples were appropriately
labeled and transferred in a cooler bag
containing ice packs to the laboratory. The
serum was then separated into plastic vials
after centrifugation and stored at 200C and
serum ferritin evaluation done later in
batches of ten.

Okoh D. A. & Nwabuko C. O., Med. Res. Chron., 2015, 3 (1), 97-101

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Estimation of serum ferritin level of a cohort of patients with sickle cell anemia in Port Harcourt, Nigeria.

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Estimation of serum ferritin level of a cohort of patients with sickle cell anemia in Port Harcourt, Nigeria.

Table 2: Mean Serum Ferritin of Transfused and Non-Transfused SCA Subjects

Parameters
No. Of Patients
No. Of Patients Never
Transfused
Transfused
41(76%)
13. (24%)
Mean Serum Ferritin (g/dl) 407200
20085
Table 3: Distribution Of Serum Ferritin Among SCA And Control Subjects
Serum Ferritin (g/L)
Percentage (%)
SCA subjects
Control subjects
< 25
(0%)
(0%)
25-300
11 (20%)
54 (100%)
300 -1000
38 (71%)
(0%)
> 1000
5 (9%)
(0%)

Okoh D. A. & Nwabuko C. O., Med. Res. Chron., 2015, 3 (1), 97-101

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Serum ferritin evaluation was done through

Result:
Spectrophotometric techniques, using the
The SCA patients had a mean serum ferritin
ferritin Latex Kit (Batch No. COD 31929
level of 300200 g/L (range 80 - 1200
Bio
systems,
Barcelona
Spain).
g/L) which was higher than the mean of
Spectrophotometry was done using the
8822 g/L (range 12-150g/L) of the
Mannheim Boehringer computerized (Rev
control group (P < 0.001). (Table 1)
371, Auto lab machine serial /No. 9510274).
The SCA patients previously transfused from
The Auto Lab machine is a selective access
this study were 76% (41).The SCA patients
batch clinical chemistry auto analyzer with
who were previously transfused also had
in-built quality control monitor. The
elevated mean ferritin level when compared
reference range for serum ferritin (15with the non-transfused patients. However,
300g/l) based on the assay method was used
there was no significant positive correlation
for the interpretation of the results.
between serum ferritin concentration and the
number of units of blood transfused (r = 0.25
Data Analysis
The data collected was analyzed using
(P = 0.061). (Table 2) About 20% (11) had
computer based statistical software (EPIadequate iron stores comparable to the
INFO VERSION 6). Primary statistics such
healthy control group; while80% (43) of the
as mean, standard deviation and correlations
SCA patients had increased iron stores.
were determined, and appropriate tests of
About 9% of the SCA patients had serum
significance such as student t-test and chiferritin above 1000 mcg/Litre. However,
square were computed. The value (p<0.001)
none of the SCA subjects in this study had
connotes statistical significant difference for
ferritin level suggestive of iron deficiency
the mean serum ferritin of SCA and Control
anemia.
(Table
3)
subjects.
Table 1: Mean Serum Ferritin of SCA and Control Subjects
Parameters
Sickle Cell Patient
Control subjects P-value
Range of serum
80-1,200
12-150
Ferritin. g/L
Mean Serum Ferritin 300200
8822
(P < 0.001)
g/L

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Discussion
The patients with sickle cell anemia in this
study were found to have higher
concentration of serum ferritin than in the
control group of same age and sex. The mean
ferritin value of the SCA patients was
comparable to the findings reported by
oluboyode et al6 in Ibadan and Ikusemoro et
al in Benin 12. Likewise, the SCA patients
who previously had blood transfusion were
found to have higher, mean ferritin level,
compared to those who were never
transfused. However, similar to the report by
Oluboyede and colleagues, there was no
correlation
between
serum
ferritin
concentration and the number of units of
blood transfused. Ikusemoro et al12 on the
contrary observed a significant correlation in
their study. This may be attributed to the fact
that more than 30% of SCA patients in their
study population had multiple transfusions,
unlike in our study where only sporadic topup transfusions were given and none was on
chronic transfusion regimen. Inadequate
transfusion record of the individuals was also
a major limitation.
Although iron over load is a clinical concern
in SCA, unlike most congenital hemolytic
anemias; it does not occur without repeated
blood transfusion 13, 14Some studies have also
shown that iron deficiency, rather than iron
over load may be a problem especially, in the
non-transfused SCD population 7. However,
in this study, none of the SCA patients had
ferritin concentration suggestive of iron
deficiency anemia; rather, increased iron
store was observed in 43 (80%) of the SCA
group with 9% having values greater than
1,000g/L but not up to 1,800g/L15 which is
level often associated with a higher risk of
organ dysfunction. Coincidentally, according
(MDS
to
international
guidelines16
symposium) the serum ferritin level above
1,000g/L represents the threshold of the
target value for the initiation of iron chelation
therapy for patients on chronic transfusion

regimen. Such patients are at risk of having

iron overload, a clinical condition known as
haemochromatosis. The major vulnerable
organs at risk in haemochromatosis are the
liver, heart, macrophages and pancreas. They
may present clinically with joint pain,
weakness, weight loss, polyuria, features of
cardiomyopathy, hence, periodic serum
ferritin assessment for SCA patients becomes
needful.
In conclusion, sickle cell anemia patients in
this environment have increased iron stores.
Consequently, iron overload may be a
problem especially for those on recurrent
blood transfusion therapy. Sequential
screening for iron overload is therefore
recommended for chronically transfused
patients. Although all the SCA patients in the
study population had adequate iron stores,
the risk of iron deficiency anemia should not
be ruled out especially, in those who have
never been transfused. However, iron
supplementation
should
only
be
recommended in proven cases of iron
deficiency anemia.
Conflict of interest:
The authors declare that they have no conflict
interest regarding this research. The research
was not funded by any organization.
Acknowledgement
The authors wish to acknowledge the
contributions of Professor A.O. Ejele of
Department of Haematology, University of
Port Harcourt Teaching Hospital, for creating
the enabling environment as well as the
secretarial staffs of the Braithwaite Memorial
Specialist Hospital, Port Harcourt, Rivers
State Nigeria.
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Estimation of serum ferritin level of a cohort of patients with sickle cell anemia in Port Harcourt, Nigeria.

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