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HEMATOLOGICAL CHARACTERISTICS IN ADULT BAHRAIN SICKLE CELL DISEASE PATIENTS

 
 

Date   :  05-03-2007

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HEMATOLOGICAL CHARACTERISTICS IN ADULT

BAHRAIN SICKLE CELL DISEASE PATIENTS

 

Shaikha Salim Al Arrayed, MBBCh, DHCG, PhD*

 

ABSTRACT

 

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.

 

Introduction

 

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.

 

 

 

TABLE 1

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

 

 

TABLE 2

 

Haematological reference range for Bahraini

Sickle cell anaemia patients

 

Parameter                 Percentile

                   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)

f/1

Mean cell Haemoglobin (MCH) in Picograms pg

Mean cell Haemoglobin concentration (MCHC)

in g/l.

Reticulocyte %.

 

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.

 

TABLE 3

 

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

 

 

Discussion

 

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.

 

References

 

1. Mohd AM. Al Hilli F, Nadkarni KV et al. Haemoglobinopathies and glucose 6 phosphate dehy­drogenase in hospital population of Bahrain. Annals of Saudi Medicine 1992: 12: 536-39.

 

2. Odenheimer DJ. Whitten CF. Ruknage DL et al. Hetrogeniety of sickle cell anaemia based on a profile of haematological variables. American Joumal of Hu­man Genetics 1983; 35: 1224-1240.

 

3. El Hazmi MAF, Warsy AS. On the nature of sickle cell disease in the south province of Saudi Arabia 1986; Acta Haemat. 76: 2V'-'l6

 

4. Weatherahl DJ. Clegg JB. Blankson J et al. A new sick­ling disorder resulting from interaction of the genes for haemoglobin S and a-thalassaemia. Br J Haemat 1969: 17: 517-526.

 

5. Steinberg MH, Rosenstock W, Colman MB, et al. The cooperative study of sickle cell disease. Effects of tha­lassaemia and microcytosis on the haematologic and vaso-occlusive severiety of sickle cell anaemia. Blood 1984; 63: 1353.

 

6. De Celaer K, Higgs DR. Weatherall DI et al. a-thalas­saemia reduces the haemolytic rate in homozygous sickle cell disease. New England Journal of Medicine 1983; 309: 189-190.

 

7. Wainscot JS. Them SL. Higgs DR et al. A genetic marker for elevated levels of haemoglobin f in ho­mozygouz sickle cell disease. British Journal of hae­matology'. 1985. 60: 261-268.

 

8. Powars DR Natural history. of sickle cell disease: The first ten years. Semin Haematol 1975: 12: 267.

 

9. Pernbrev ME. Wood WG. Weatherall DJ et aI. Foetal haernoa!obin Production and sickle gene in the oasis of Eastern Saudi .Arabia. BrJ Haematol 1978: 40: 415.

 

10. Miller BA. Salameh M. Ahmed M at al: High hemoglobin prcduetion in sickle cell anaemia in the Eastern province of Saudi Arabia is genetic all; determined. Blood 1986.5Th 1405-1410.

 

11. Weatherall DJ. Clegg JB. Wood WG. The hemoglobin in Scriver CR. Beandet AZ. Sly WS. Vaileds. The meta­bolic basis of .inherited disease 6th ed 1988. New York. Mc Craw HI Book Co.

 

12. Emburv SH. Clark MR. Monrov G & Mohandas N. Concurrent sickle cell anaemia and alpha thalassaemia. Effects on Pathologic al properties of sickle erythro­cytes Journal of clinical investigation 1980; 73:116 -123.

 

13. Dover GJ Gharsh S & Heintzelman k. Individual vari­ation in the production and survival of F cells in sickle cell disease. New England Journal of Medicine 1978:299: l428 -35

 

14. Arrayed SS. Haematological values among Bahraini -in press.

 

15. Refastham RD. Clinical Haematologv 1984, sixth edi­tion, Wright. Bristol: 7: 91-98.

 

16. Modell M, Berdoukas V. Clinical approach to Thalas­saemia. Grune 7 Stratton 1984; 354-383.

 

17. Serjeant CR. Foster K. Serjeant BE. Red cell sire and the clinical and haematological features of homozygous sickle cell disease. Br Journal Haematol 1981; 48:445.9.

 

18. El-Hazmi MAF. Haemoglobin disorders: A pattern for thalassaemia and haemoglobinopathies in Arabia. Acta Haemat 1982: 68: 43-5 1.

 

19.         Higgs DR. .Albridge BE. Lamb Jet al. The interaction of alpha thalassaemia and homozygous sickle cells dis­ease. New England Journal of Medicine 1982. 306:1441-1-146.

 

20.         Perrine RP. Brown MJ. Clegg JB et al. Benign sickle cell anaemia. Lancetll 1972; 1163-1167.

 

21.         El Hazmi NIAF, Jabber FR, A1-Faleh FZ. The haematoloaical. biochemical and clinical expression. Trop Geogr Med 1987; 39: 157-162.

 

22.         El-Hazrni MAF. Clinical manifestation and laboratory findings of sickle cell anaemia in association with a­thalassaemia in Saudi Arabia. Acta Haemat 1986; 74:155- 160.

 

23.         El-Hazmi MAE Bahakim HM. Al Swalem AM. Warsy AS. The feature of sickle cell disease in Saudi chil­dren. J Trp Paed 1990; 73: 31-34.

 

24.         Babiker M.A. Taha SR. Two different patterns of sickle cell disease in children in Saudi Arabia. Ann Trop Paedr

1982; 2: 179-SI.

 

 

 
     
 

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