BMC Hematology

Background: Deficiency in coagulation factor VIII encoded by F8 results in the X-linked recessive bleeding disorder haemophilia A (HEMA). Here we describe the identification of a novel variant in the factor VIII gene, F8 , in an adult male patient with severe haemophilia A. Case presentation: The patient was diagnosed in early childhood and subsequently co-infected with Hepatitis C and HIV acquired during early blood transfusion for haemophilia in the 1980ies. The identified F8 deletion, c...

Background: An insight into the utilization pattern helps in future planning of blood drive. This study was conducted to describe the demographic characteristics of the transfusion recipients and pattern of blood and blood product utilization in Nigeria. Methods: Blood bank registers of University of Calabar Teaching Hospital (UCTH) Calabar were analysed for a 12 month period. Number of blood units requested, number of units issued, Cross-match to transfusion ratio (C/T), age, gender, blood group, blood components received, patients ward and clinical diagnosis were computed...

Background: In pregnancy, hematological changes occur in order to meet the demands of the developing fetus and placenta, with major alterations in blood volume. Abnormal hematological profile affects pregnancy and its outcome. This study aimed to assess hematological profiles of pregnant women at a tertiary care teaching hospital. Method: This cross sectional study was conducted among 284 consecutive pregnant women at St. Paul's Hospital Millennium Medical College...

Background: Anemia affects a significant part of the population in nearly every country in the globe. Iron requirements are greatest at ages 6-23 months when growth is extremely rapid and critically essential in critical times of life. Even though infants and toddlers are highly at risk, they are not considered as separate populations in the estimation of anemia. Despite this, the prevalence of anemia among under 24 months of age is still at its highest point of severity to be a public health problem in Ethiopia...

Background: Evidence-based guidelines for sickle cell disease (SCD) health maintenance and management have been developed for primary health care providers, but not for individuals with SCD. To improve the quality of care delivered to individuals with SCD and their caregivers, the main purposes of this study were to: (1) understand the desire for patient-centered guidelines among the SCD community; and (2) adapt guideline material to be patient-centered using community-engagement strategies involving health care providers, community -based organizations, and individuals with the disease...

Background: In 1962 Victor Herbert developed megaloblastic anaemia four months after commencing a severely folate-deficient diet whereas, in his self-experiment 50 years later, this author took 19 months to fully deplete his liver folate store. This author proposed that his own larger initial liver folate store, due to his vegetarian diet and consumption of fortified foods, was the cause of the time difference. Main text: This author now proposes that Herbert was also likely to have been deficient in vitamin C, thus shortening the time taken to develop folate deficiency...

Background: Diabetes mellitus is a very rampant metabolic disorder, particularly type II. It has many complications such as the septic foot. Diabetic septic foot (DSF) patients are at high risk for coagulation abnormalities as well as surgical hazards. Owing to the potential sequelae of coagulation and vascular abnormalities, this work aimed at studying the hemostatic state and platelet indices in diabetes type II patients with septic foot. Methods: A case-control study was conducted during the period from July to September 2017 at Dr...

Blood transfusions are often essential for treatment of severe anaemia and pregnancy complications. The unavailability of blood is a medical concern, especially in developing countries. New sources of red blood cells (RBC) are under investigation. Several studies have attempted to produce functional RBC from CD34+ haematopoietic stem cells (HSC) isolated from peripheral blood and umbilical cord blood, from embryonic stem cells (ESC) and induced pluripotent stem cells (iPSC). A recent article published in Nature Communications describes a novel model for generating RBC from a stable erythroid cell line obtained from bone marrow CD34+ haematopoietic stem cells (HSC)...

Background: Hematological abnormalities are common in HIV positive patients. Of these, thrombocytopenia is a known complication which has been associated with a variety of bleeding disorders. However, its magnitude and related factors have not been well-characterized in the era of highly active antiretroviral therapy (HAART) in Ethiopia. Therefore, this study aimed to assess the prevalence of thrombocytopenia before and after initiation of HAART among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia...

Background: In individuals infected with HIV, hematological abnormalities are common and are associated with increased risk of disease progression and death. However, the profile of hematological abnormalities in HIV infected adult patients is not known in Ethiopia. Thus, the aim of this study was to assess the hematological manifestations of HIV infection and to identify the factors associated with cytopenias in both HAART and HAART naïve HIV infected adult patients in Ethiopia. Method: We conducted a cross-sectional quantitative study of HIV-infected adult patients attending the ART follow-up clinic of Jimma University Specialized Hospital in Jimma, Ethiopia, from July 2012 to September 2012...

Background: Anemia is the most common hematological abnormality in Human immunodeficiency virus (HIV) positive patients and a significant predictor of its progression to AIDS or death. This study was aimed to assess the prevalence of anemia before and after initiation of antiretroviral therapy (ART) among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia. Methods: A cross sectional study was conducted from January to April, 2017 in Black Lion Specialized Hospital, Addis Ababa, Ethiopia...

Background: Immunophenotypic analysis of the bone marrow (BM) cells has proven to be helpful in the diagnosis of Myelodysplastic Syndromes (MDS). However, the usefulness of flow cytometry (FCM) for the detection of myelodysplasia in the peripheral blood (PB) still needs to be investigated. The aim of this pilot study was to evaluate the value of FCM-based PB neutrophil and monocyte immunophenotyping for the diagnosis of lower risk MDS (LR-MDS). Methods: We evaluated by 8-color FCM the expression of multiple cell surface molecules (CD10, CD11b, CD11c, CD13, CD14, CD15, CD16, CD34, CD45, CD56, CD64 and HLA-DR) in PB neutrophils and monocytes from a series of 14 adult LR-MDS patients versus 14 normal individuals...

Background: Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia...

Myelodysplastic syndromes (MDS) encompass a diverse group of hematologic disorders characterized by ineffective and malignant hematopoiesis, peripheral cytopenias and significantly increased risk of progression to acute myeloid leukemia (AML). The hypomethylating agents (HMA) azacitidine and decitabine induce meaningful clinical responses in a significant subset of patients with MDS. Though never compared directly with decitabine, only azacitidine has improved overall survival (OS) compared to conventional care in a randomized trial in patients with higher-risk MDS...

Background: 5-Azacitidine administered as a 7-day dosing regimen (7-0-0) is approved in high risk IPSS myelodysplastic syndrome (MDS) patients. Alternative regimens such as a 5-day (5-0-0) or 7-day with a weekend break (5-2-2) are commonly used. No randomized controlled trial has been done directly comparing all three dosing regimens. The objective of this study was to compare the efficacies of the 5-0-0, 5-2-2, and 7-0-0 regimens in MDS and AML. Methods: A systematic review was conducted using MEDLINE, EMBASE and CENTRAL...

Background: Information on the hemoglobin status of pregnant and lactating mothers was scarce. The objectives of this study were to determine the burden and determinants of anemia in the pregnant and lactating mother. Methods: A comparative cross-sectional study was conducted. Descriptive statistics were used to identify the prevalence of anemia. Binary logistic regression and multiple linear regressions were used to identify the predictors of anemia. Results: The prevalence of anemia in lactating and pregnant women was 43...

Background: Vascular thrombosis is an important pathophysiological aspect of sickle cell disease (SCD). This study aimed to investigate the prevalence and clinical impact of factor V Leiden G1691A (FVL) and prothrombin G20210A mutations among Palestinian sickle cell disease (SCD) patients. Methods: A total of 117 SCD patients, including 59 patients with sickle cell anemia (SS), 33 patients with sickle β-thalassemia and 25 individuals with sickle cell trait (AS) were studied...

Background: Abnormal fibrinogens can be caused by clinically silent hereditary mutations. A new case was detected accidentally in an 11-year-old girl when routine pre-operative coagulation tests were performed for nasal turbinate surgery. Methods: The fibrinogen genes FGA, FGG and FGB were sequenced using standard protocols. The kinetics of fibrin formation were followed by turbidity at 350 nm. Purified fibrinogen was incubated with plasmin, and the degradation products analyzed by SDS/PAGE...

Background: Hypertension is a major health problem worldwide. It can lead to cardiovascular disease and also leads to functional disturbances including hematological parameters. The abnormalities of haematological parameters may enhance an end-organ damage. Therefore, the aim of this study was to assess some hematological parameters of hypertensive individuals in comparison with normotensive individuals at University of Gondar hospital, northwest Ethiopia. Methods: A cross sectional comparative study was conducted from October to November 2015 on a total of 126 hypertensive and 126 normotensive individuals at University of Gondar Hospital...

Background: Blood can save millions of lives. Even though people do not donate blood regularly, there is a constant effort to balance the supply and demand of blood. The aim of this study was, therefore, to determine the knowledge, attitude and practice of blood donation between university students. Methods: The comparative cross sectional study design was used in Adama Science and Technology University and Arsi University from April 11-May 2, 2016.360 students were selected using stratified sampling...