HEMATOLOGICAL CHARACTERISTICS IN ADULT
BAHRAIN SICKLE CELL DISEASE PATIENTS
Shaikha Salim Al Arrayed, MBBCh, DHCG, PhD*
تعرض هذه الدراسة وصفا دقيقا لحالة الدم لدى المرضى البحرينيين المصابين بفقر الدم المنجلي حيث جمعت وحللت خمسون عينة دم من مرضى بحرينيين مصابين بفقر الدم المنجلي تراوحت اعمارهم بين 15 و 50 عاما.
وقد وجد أن الهيموغلوبين أقل من 10 ملغ/دل لدى 60% من المرضى بينما ارتفع مستوى الهيموغلوبين الى 12 ملغ/دل لدى 8.8% من هؤلاء المرضى . كما تبين ان ال HCT يقل عن الثلاثين لدى 57% من المرضى كما كان الـ MCH دون الـ 25 ( Pg ) لدى 64% أما الـ MCV فقد قلت عن FL 67 لدى 62% مما يدل على صغر الكريات الحمراء.
كذلك وجد أن الصورة الدموية كانت متسقة مع الشكل غير لبحاد من فقر الدم المنجلي.
This is a study of the hematological picture of Bahraini sickle cell disease patients. Blood samples from 50 Bahraini patients with Sickle Cell Disease (SCD) were collected and analyzed. The age of these patients ranged from 15-50 years. We found that 60% of the patients have HB lower than l0 gm/dl, and that only 8.8% have HB above l2gm/dL. 57% of these patients have HCT below 30. 64% have MCH below 25pg. 62% have MCV below 76fl which indicate microcytosis. We also found that the blood picture is consistent with the mild form of the disease.
It is well known that the three major types of hemoglobinopathies are found in Bahrain', and many different combinations of hemoglobinopathies genes are expected. All may occur with or without the coincidental G6PD deficiency. These complex interactions are likely to produce a spectrum of severity in the clinical and in the hematological picture2.
Interaction of the alpha thalassemia gene with the SCD influences the hematological indices
as it causes microcytic hypochromic anaemia. It reduces the hemolytic rate and increase deformity of red ce11s31. On the other hand the inheritance of a heterocellular persistence of fetal hemoglobin by a patient with SCD may result in very high levels of HbF and it is widely accepted that this condition is associated with mild clinical picture and haematological course 8 - 3.
Materials and Methods
The aim of this study is to find the haematological status for the Adult Bahraini sickle cell anaemia patients. Retrospective cases were taken up at random from the record of the haematology section of the pathology department, over a period of 3 months, starting January 1991 to.
Comparison between haematological values
for Bahraini Sickle cell anaemia patients,
and those for normal Bahraini
Parameter Mean SD SE Normal
Hb 100 1.5 .22 141.7
WEG 10.62 5 .67 6.67
REC 4.09 .75 .11 5.03
HCT 29.7 4 .6 42.1
MCV 74.4 11 1.6 82.98
MCH 24.9 .4 .6 27.8
MCHC 33.4 1.4 .6 33.3
Retics 6.87 5.4 .7
ROW 17.1 2.9 .44
HB F 13.4 6.5 1.2
Haematological reference range for Bahraini
Sickle cell anaemia patients
5 50 95
7.8 9.8 13.0
W8C 4.9 9.2 19.8
REC 2.7 . 4.2 5.8
PCV 23.9 29.0 38.0
MCV 59.5 72.0 98.0
MCH 18.8 23.9 33.0
MCHG 31.0 33.3 36.0
Retics 1.0 6.0 19.0
ROW 13.5 16.7 22.7
HBF 1.0 12.6 25.2
March 1991. A total of 50 cases of Bahraini sickle cell anaemia patients all of them were in a steady state, were collected. The age of these patients ranged from 15-50 years. 2m1 of blood were collected from each in anticoagulant tubes containing 3mg of Disodium salt of Ethyline Diamine Tetra Acetic Acid. The blood samples were subjected to routine haematological analysis in Coulter S plus IV automated counter which did the counts by electrical impendance method under strict internal quality control with the help of Coulter 4C controls, and UK national external quality control schemes.
The following parameters in their respective 5.1. units were taken up for the statistical study:
Haemoglobin (Hb) in gram/ deciliter (gm/dl).
White blood cell (WBC) in numbers/1.
Red blood cell (RB C) in numbers /1.
Packed cell volume (HCT,PCV) in 1/1.
Mean cell volume (MCV) in (Femtoliter/cell)
Mean cell Haemoglobin (MCH) in Picograms pg
Mean cell Haemoglobin concentration (MCHC)
Information was stored in an IBM compatible personal computer using Data Base 111 plus software, and the data were analyzed using SPSS software. The statistical method used for basic haematological results were the arithmetic mean, standard deviation, standard error of the mean, together with the distribution curve for each parameter.
Results of the study
Table 1 shows the haematological parameters of 50 sickle cell anaemia patients with the mean, standard deviation, standard error of the mean, and the comparison with the haema~ological values for normal Bahraini.
Table 2 shows the frequencies and the reference limit which is defined as the 5, 50, 95 percentile.
Table 3 shows the comparison of the haematological values in SCD patient from Bahrain,
Eastern province of Saudi Arabia, Western province of Saudi Arabia.
Fig 1 shows the distribution curves for all the parameters.
Comparison between haematological values
for Bahraini SCD disease patient and
patient from the Eastern and
Western Province of Saudi Arabia
Parameter Bahraini Eastern P Western P
Hb 100.0 ± 1.5 108.0 ± 1.5 84.0 ± 1.5
RBC 4.8 ± 0.75 3.9 ± 0.9 3.0 ± 0.8
HCT 22.7 ± 4.0 30.0 ± 0.59 23.0±0.05
MCV 74.4 ± 11.0 78.5 ± 10.0 81.3 ± 12.8
MCH 24.9 ± 0.4 28.6 ± 5.1 29.0 ± 5.6
MCHC 33.4 ± 1.4 36.1 ± 3.6 36.1 ± 5.22
Retics 6.9 ± 5.4 6.5 ± 4.2 21.6 ± 10.3
H8 F 13.4 ± 6.5 11.3 ± 6.2 10.3 ± 7.0
Comparing the haematological values for Bahraini SCD patients and these values for normal Baliraini'4, we found that the SCD patients have low values in all the parameters studied (Table 1).
Studying the distribution curves (Fig 1), we found that 60% of the patients have Mb Lower than 10 grn/dL, and that only 8.8% have Hb above 12 gm/dL. The normal Hb for adult is 12 grn/dL or above it'5.
57% of these patients have HCT below 30. 64% have MCH below 25 pg. while the normal level of MCH is 28 pg or above. The low level of MCH in these patients is partly due to presence of thalassaemia gene and partly due to iron deficiency anaemia. Modell 1984 suggested that all those with MCH below 27 should be tested for thalassaemia or iron deficiency anaemia.
MCV is also shown to be on the lower side as 62% have MCV below 76 fl which indicates microcytosis which is partly due to the coexistence of Alpha thalassaemia gene or due to iron deficiency anaemia'7.
MCHC is nearly normal as 57% have MCHC in the range of 32-34 gmn/dL.
These patients show a high reticulocyte count as 76% have a count more than 3%.
Alpha Thalassaemia genes in HbS heterozygotes modify the haematological parameters. The effect is mainly on the level of MCV, MCH, and the Alpha/Beta ratio 17.21. The influence of alpha thalassaemia gene on the haematological presentation was investigated in Saudi patients with one and two gene deletions~. The results were compared to the results obtained in SCD patients without alpha thalassaemia and in SCD patients with 2 gene deletion. The mean cell volume, mean cell haemoglobin, and HbF were significantly lower in the second group, while packed cell volume and Hb A2 were significantly higher than in the SCD patient without alpha thalassaemia. RBC count and haemoglobin were higher in the former group. but the difference is not statistically
significant. Patient with one gene deletion have intermediate values. With the high prevalence rate of alpha thalassaemia among Bahraini which is 24%, we expected to have similar effect of alpha thalassaemia on the haematological picture of our SCD patients.
Another study was done in Saudi Arabia comparing the haemato logical values in SCD patients from the Eastern province and those from Western province23. These two groups were found to have different haplotypes. The Asian haplotype predominate in the Eastern patients while the African haplotype. Benin type or S1 predominate in the Western province. There were significant difference in the total haemoglobin. red blood cells, and haematocrit values, while the red cell indices, i.e.. mean cell volume, mean cell haemoglobin concentration, and the percentage of HbF did not show any significant difference. When we compared the Bahrain patient values with these two groups, we found that the Bahraini are similar to those from the Eastern province of Saudi Arabia.
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