International Blood Research & Reviews

The therapeutic benefits of camel milk consumption are a supplement to routine sickle cell disease management. In maintaining hemolytic crises in sickle cell anaemia, patients were assessed during a six weeks study. Throughout the study, 20 patients were recruited for the study and divided into 4 groups, 5 patients per group. Group 2, 3 and 4 were treated with daily consumption of raw camel milk (100 ml, 50 ml + Folic acid + Paludrin and 100 ml + Folic acid + Paludrin respectively). In all groups, the foetal haemoglobin (Hb F), packed cell volume (PCV), platelet, red blood cell (RBC) and white blood cell (WBC) count were measured before initiation of the study and monitored at 2 weeks intervals for 6 weeks. In the group that took camel milk (50 ml in addition to Folic acid and Paludrin), there was a significant increase in WBC (8.16 ± 4.12 to 16.68 ± 3.53), a significant increase in PCV (21.28 ± 1.23 to 25.24 ± 1.11) with decrease in platelet (311.80 ± 61.93 to 260.40 ± 29.22) and significant increase in Hb F (7.06 ± 2.42 to 10.02 ± 2.41) compared to group 1 (control). However, there was no significant difference in the haematological parameters of group 2 and 4. The results implied that the consumption of camel milk in sickle cell patients resulted in an increase in foetal hemoglobin concentration which prevented crises in almost all the patients. Increase in foetal haemoglobin has been postulated to reduce hemolytic crises in sickle cell anaemia patients. Based on these findings, camel milk consumption may, therefore, be considered useful in the management of sickle cell diseases.

Sickle cell disease is a global public health problem. L-arginine is an amino acid that helps in improving blood in the arteries of the heart and improved symptoms of clogged arteries, chest pain or angina and coronary arteries disease. Nitric oxide is a powerful neurotransmitter that helps blood vessels relax and improve circulation. The l-arginine and nitric oxide levels of sickle cell disease (SCD) subjects with steady stages were also significantly low. The objective of this study was to evaluate L-arginine and Nitric oxide levels in children with sickle cell disease at steady state for 8 weeks.  This study included children with a confirmed HbSS electrophoretic pattern aged 1-14 years presented to the sickle cell clinic unit of Federal Teaching Hospital Gombe. The L-arginine and nitric oxide levels were significantly higher post supplementation compared to baseline levels (p = 0.002 and 0.000 respectively). It is recommended that L-arginine supplementation be included in the management of patients with sickle cell disease. L-arginine supplement should be made available in the paediatric emergency unit, clinic and pharmacy department as given to patients with sickle cell disease to prevent the adverse effects during the crisis and potentially reduce the length of stay in the hospital.

Chronic myelogenous leukaemia (CML) is an acquired myeloproliferative disorder (MPD) characterized by a chromosomal abnormality (the Philadelphia chromosome) that causes the chimeric BCR-ABL oncogene. An acquired genetic mutation in exon 12 of the JAK2 tyrosine kinase gene leading to a substitution of a valine for a phenylalanine (V617F) has been described as the most common form of CML for those who test negative for the Philadelphia (Ph) chromosome. According to World Health Organization (WHO) classifications (2008), the JAK2 V617F mutation and the BCR-ABL translocation are mutually exclusive for Ph(-) and Ph (+) MP, respectively. We studied the JAK2 V617F mutation in Ph+ myeloid leukaemia in a cohort of 27 Beninese patients. The ARMS multiplex PCR technique was used to identify the JAK2 V617F mutation in all patients. Most of the patients were diagnosed as in the chronic phase (88.9%) of the disease, and all of them were carriers of the Philadelphia chromosome and considered Ph (+). No patients with the BCR/ABL translocation carried the JAK2 V617F mutation. JAK2 V617F is specific to Philadelphia gene negative MP.

Introduction: A Transfusion transmitted infection (TTI) is a virus, parasite, or other potential pathogen that can be transmitted in donated blood through a transfusion to a recipient. This transmission is one of the most dreaded complications of blood transfusion.

Aims and Objectives: The present study is aimed at evaluating the prevalence of TTIs among the blood donors in Madhya Pradesh, a central state of India 

Materials and Methods: The present five years (1^st January 2012 to 31^st December 2016) study is a retrospective analysis of one million three hundred thirty-six thousand one hundred fifty-six (1,336,156) blood donor’s record at Madhya Pradesh Aids Control Society (MPSACS) Bhopal; donors donated their blood at National Aids Control Organization (NACO) supported blood banks, Madhya Pradesh, India. With the permission of MPSACS, donor’s data was collected, retrieved, tabulated, summarized and compared statistically by frequency distribution and percentage proportion. Chi-square (X²) test was applied to evaluate the significant (p-value) ratio of difference statistically.

Results: In the present study, 91.1% were voluntary blood donors and rested 8.9% replacement blood, donors. Male to female ratio of blood donors were male 94% and female 6%. TTIs prevalence in the study was 1.75 % (p<0.001) while individual infections prevalence was; HbsAg 1.16, Syphilis 0.37%, HCV 0.09%, HIV 0.08%, and Malaria 0.04%.

Conclusion: Low prevalence of TTIs was reported in the present study, so hereby we have concluded that a healthy transfusion service is must for the health care system of the state/country. 100% Voluntary blood donation by Regular, voluntary, non-remunerated blood donors from low-risk populations and Careful TTIs testing, preferable NAT to reduce the window period are the key factors to ensure the safe blood to the needy.

Aim: To highlight the diagnosis of haemophilia B in 5 Nigerian brothers as well as the diagnostic and management challenges inherent in resource-poor settings.

Presentation of Cases: We report the cases of 5 brothers with ages ranging from 2 years to 13 years seen at the paediatric out-patient unit of the Federal Medical Centre Azare, Nigeria. They presented with complaints of abnormal and excessive bleeding since the neonatal period. Bleeding was often provoked by events ranging from traditional uvulectomies, dental exfoliation to circumcisions and was severe enough to require blood transfusions in some instances. Following diagnosis, genetic counseling was offered to the family and plans put in place to commence administration of prophylactic factor IX concentrate.

Discussion: The peculiarity in these cases is the large number of affected individuals in one family. This is made more remarkable by the fact that haemophilia B (HB) is extremely rare in Nigeria. The reason for the relative rarity of HB in Nigeria is not known. However, it is recognized that the genetic mutations associated with HB are diversely distributed and often show variations between and across ethnic groups. This may account for the spread and variability in clinical manifestations of the disease. 

Conclusion: Haemophilia B though very rare may cluster in individual families. The unavailability, as well as the high cost of coagulation factor concentrates in resource-poor settings remains a significant challenge for physicians and patients alike.