Several reports indicate that haemoglobinopathies and thalassemias are the most common genetic abnormalities in the population of the Arabian- Peninsula. However the exact frequencies of these abnormalities among the Qatari population has not yet been determined. We surveyed the results of all hemoglobin-electrophoresis performed in Hematology laboratory at Hamad Hospital over a period of 78 months (Jan.1994- June.2000) together with the results of all other relevant tests to highlight the distribution of these disorders among the Qatari patients seen at Hamad Hospital. The size of the group analyzed was 3275 of whom 1702 were Qatari and 1573 were non-Qatari residents of different nationalities. The survey of Qatari results revealed that 30.43% of all Qatari patients tested carry fi-thalassemia (minor, intermedia or major), 12.28% carry oc-thalassemia (minor or intermedia) while 0.53% were labeled as having Hereditary Persistence of Fetal Hemoglobin (HPFH). fithalassemia is the most common type of thalassemic disorders seen in the Qatari patients, accounting for 71.25% of all thalassemias encountered in this group. Out of Qatari patients tested 16.33% were found to carry a structurally abnormal hemoglobin variant of one type or another. Sickle cell hemoglobin (Hb-s) is the most common structural hemoglobin variant detected where it is found in 14.63% of those tested and constituting about 83.97% of all structural hemoglobin variants detected. Hb.D was found in 1.41% of those tested and constituted 12.42% of all structural hemoglobin variants detected in this group. Other structurally abnormal hemoglobin variants such as Hb. E and Hb. C were less common. As expected, almost all possible combinations between different types of structural hemoglobin variants and different types of thalassemias could be identified. Considering the fact that the hematology laboratory at Hamad Hospital is the only laboratory in the State of Qatarthat performs hemoglobin- electrophoresis we hope that this study will reflect for the first time a reasonable idea about the prevalence of thalassemia and other types of hemoglobinopathies among the indigenous Qatari population and help to provide a comprehensive baseline information for any proper future epidemiological studies to establish the exact frequencies of these genetic disorders among Qatari nationals and for any future molecularbased studies for elucidation of the molecular basis of these disorders in this population.
A total of 78,428 blood units collected by the Blood Donor Unit, Hamad Medical Corporation, Doha, from the multinational donors of Qatar (28,622 Qatari nationals donors and 49,806 Non-Qatari donors) in the period January 1994 to Dececember 2001 were screened for hepatitis markers. About 10,382 units (13.2%) were discarded because of positivity for one or more hepatitis markers; 769 units (0.9%) were positive for hepatitis-B surface antigen (HBsAg), 8516 units (10.9%) were positive for hepatitis-B core antibodies (HBcAb) and 1097 units (1.39%) were positive for hepatitis C (HCV) antibodies. There was no significant difference between the rate of positivity for HBcAb and HBsAg in indigenous Qataris and Non-Qataris despite a slightly higher rate ofpositivity for HCV antibodies in the Non-Qatari group. An outstanding finding was the significantly high rate of positivity for HCV antibodies in Egyptian donations (11.2%), which accounted for 31.2% of all discards for the Non-Qatari group. As the Blood Donor Unit at HMC is the only blood collection center in Qatar it is assumed that the results could be representative of the country as a whole.
Three adults with sickle cell disease (SCD) presented in the span of less than two years with pancytopenia, signs of Disseminated Intravascular Coagulation (DIC) and no localizing pain to suggest vaso-occlusive crisis (VOC). Bone marrow examination showed almost complete necrosis of the whole series of marrow cells, thus explaining the pancytopenia. Some necrotic leukocytes were noted also in the peripheral blood. The condition was self-limiting with marrow regeneration occurring in all three patients after transfusions of red cells and platelets.
The reference range for Haemoglobin A2 (HbA2) by the microcolumn technique is not well defined. Various textbooks and inserts give different reference ranges. This leads to confusion in interpretation. Normal Saudi individuals were checked by the method, excluding all disease in them that can affect HbA2 level. The reference range obtained was 2.0-2.7 (mean+SD). This new range will decrease remarkably the overlap between normal, alpha thalassemia trait and beta thalassemia trait individuals. Also>, and in spite of the claim by the manufacturer to the contrary, it was shown that presence of sickle haemoglobin, places the assay of Hb A2 significantly outside the normal range> using a column that is supposed to be just for the purpose of accurate assay of Hb A2 in presence of HbS. The range in sickle trait patients was shown to be 2.5-3.4 (mean+SD).
Howell-Jolly bodies (HJB) are composed ofDNA which will apparently take the fluorescent dye used to stain the chromatin bodies of the malarial parasites (MP). While evaluating a capillary tube/fluorescent staining technique for malaria we came across this previously unreported false positive effect of HJB in ten cases, all negative microscopically for MP and not originally requested for that test All gave positive results, the degree of which correlated well with the frequency of HJB. The positivity concentrated in the upper red cell layer of the capillary tube where MP rings also concentrate.
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