BMC Hematology

BACKGROUND: Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case. CASE PRESENTATION: We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension...

BACKGROUND: Circumcision in hemophiliacs is a delicate surgery because of bleeding risks that could be avoided by adequate substitution of coagulation factor. This practice is very challenging in countries where anti hemophilic treatment is inaccessible. The study aimed to evaluate a circumcision protocol in hemophilia A using low quantities of factor concentrates. METHODS: This prospective study included 26 hemophiliacs A who underwent circumcision in 2014. Medical treatment protocol using low quantity of factor concentrates was drafted by physicians of the Hemophilia Treatment Center and the surgical protocol by experienced surgeons...

BACKGROUND: Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon...

BACKGROUND: Africa is the most affected continent with 200,000 new born affected by sickle cell anemia annually with of 5% of under five deaths. Nigeria has the largest sickle cell gene pool in the world with about 2% of all babies born to Nigerian parents. This study therefore sets out to assess the prevention practices influencing the frequency of occurrence of vaso-occlusive crisis among patients in Ogun State. METHODS: This study is a descriptive cross-sectional study conducted in Abeokuta South Local Government Area Ogun State...

BACKGROUND: Familial and acquired thrombophilia are often etiologic for idiopathic hip and jaw osteonecrosis (ON), and testosterone therapy (TT) can interact with thrombophilia, worsening ON. CASE PRESENTATION: Case 1: A 62-year-old Caucasian male (previous deep venous thrombosis), on warfarin 1 year for atrial fibrillation (AF), had non-specific right hip-abdominal pain for 2 years. CT scan revealed bilateral femoral head ON without collapse. Coagulation studies revealed Factor V Leiden (FVL) heterozygosity, 4G/4G plasminogen activator inhibitor (PAI) homozygosity, high anti-cardiolipin (ACLA) IgM antibodies, and endothelial nitric oxide (NO) synthase (eNOS) T786C homozygosity (reduced conversion of L-arginine to NO, required for bone health)...

BACKGROUND: Sickle cell anemia patients suffer from oxidative stress due to chronic inflammation and self-oxidation of sickle hemoglobin (Hb S). Chronic oxidative stress contributes to endothelial dysfunction, inflammation and multiple organ damage in sickle cell disease (SCD). Thus, antioxidant medication may favorably influence the disease. Gum Arabic (GA), edible, dried, gummy exudates from Acacia Senegal tree, has been claimed to act as an anti-oxidant and cytoprotective agent, protecting against experimental hepatic, renal and cardiac toxicities in rats...

BACKGROUND: Malaria and human immunodeficiency virus are the two most devastating global health problems causing more than two million deaths each year. Hematological abnormalities such as anemia, thrombocytopenia and leucopenia are the common complications in malaria and HIV co-infected individuals. The aim of this study was to determine the effect of malaria infection on hematological profiles of people living with HIV attending Gambella Hospital ART clinic, Southwestern Ethiopia. OBJECTIVE: To determine the effect of malaria infection on hematological profiles of people living with HIV attending Gambella Hospital ART clinic, Southwestern Ethiopia...

BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most common leukemia in Western countries. The frequency of symptomatic central nervous system (CNS) involvement is unknown but thought to be a rare phenomenon. Currently there are no known risk factors for CNS involvement. CASE PRESENTATION: We describe a clinically staged low-risk CLL case that presented with symptomatic CNS involvement and progressed rapidly to death. Evaluation of the surface adhesion molecules identified a markedly altered expression pattern of the integrin, CD49d, and the tetraspanin, CD82, in the index case when compared to similar low-risk CLL cases...

BACKGROUND: Anemia is a strong predictor of mortality and poor quality of life among persons with either renal impairment or HIV infection. In this study, we investigated the prevalence of anemia and its association with renal insufficiency among HIV infected patients initiating ART at a hospital in Northeast Ethiopia. METHODS: In this retrospective cohort study, records of 373 patients on ART were selected in Dessie Referral hospital, South Wollo, Northeast Ethiopia from September 2010 to August 2013...

BACKGROUND: In Madagascar, the last study on sickle cell disease (SCD) was done in the early 1980s. The country is known as endemic for malaria and respiratory infections. The main objective of this study was to estimate the prevalence of SCD; the secondary objective was to evaluate its association with malaria and respiratory infections. METHODS: This is a cross-sectional study which was carried out in a rural village in the south east coast of Madagascar between May 2011 and November 2013...

BACKGROUND: Anaemia is one of the main factors in the deferral (disqualification) of blood donors following haematological screening. There is paucity of data on the prevalence of anaemia in blood donors in Sub-Saharan Africa. This study was undertaken to determine the prevalence of anaemia and its association with month and blood phenotype in blood donors in Fako division of Cameroon. METHODS: Blood donors were recruited between the 1st of January and 31st of December 2014, and their haemoglobin concentration (Hb) was determined using a haemoglobinometer...

BACKGROUND: Hemophagocytic lymphohistiocytosis in adults is often secundary to an infection or a neoplasm. In this last case, T cell lymphomas are the most frequent causes. Hemophagocytic lymphohistiocytosis secundary to a B cell lymphoma has been rarely reported. CASE PRESENTATION: We describe a case of a hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma treated with conventionnal chemotherapy leading to a complete remission. CONCLUSION: Prompt etiologic diagnosis and treatment of hemophagocytic lymphohistiocytosis leads to satisfactory outcome...

BACKGROUND: Transfusion-transmitted infections threaten the safety of patients requiring blood transfusion, which in turn imposes serious challenges for the availability of safe blood products that are still affordable in health care systems with limited resources. The aim of the study was to determine the prevalence of transfusion-transmitted infections in blood donors and to evaluate the demographic characteristics of reactive and non-reactive blood donors. METHODS: A prospective cohort study was conducted at our institute in Karachi, Pakistan...

BACKGROUND: When availability and/or affordability of anti-hemophilic factor concentrates are limited, optimal prophylaxis regimens in severe hemophilia A (HA) remain to be determined. In selected situations, low-dose daily prophylaxis (LDDP) may be an effective and economical option. The goal of our study was to evaluate if subjects on a LDDP regimen could achieve adherence and good clinical outcome. METHODS: Seventeen subjects (age between 15.2 and 28.4) on LDDP suffering from severe/moderate HA were followed prospectively for 2 to 3 years as part of a health-related quality of life (HRQoL) study...

BACKGROUND: Since the discovery of specific histocompatibility, literature has associated genes involved in the immune response, like the Human Leucocyte Antigen (HLA), with a better prognosis in transplantation. However, other non-HLA genes may also influence the immune process, such as the genes encoding the immunoglobulin-like receptors of natural killer cells (KIRs). The discovery that NK cell KIR receptors interact with conservative epitopes (C1, C2, Bw4) presented in HLA class I molecules that are genetically polymorphic, also observed in KIR genes, led to the investigation of the relevance of the KIR system to hematopoietic stem cell transplant...

BACKGROUND: Reasons for the variation in response of deep vein thrombosis (DVT) to anticoagulation treatment are not known. Some patients develop complications such as post-thrombotic syndrome or recurrent DVT but others make a full recovery. The aim of the study is to identify the level of variation in response to anticoagulation treatment and provide more precise and quantitative disease characterisation in response to treatment. METHODS: A prospective observational study using duplex ultrasound to examine changes in thrombus characterisation, evolution and resolution over a 2 year period in patients with a confirmed DVT...

BACKGROUND: Although the demand for blood supply has progressively increased in developing countries, evidences indicate that there is a major shortage of blood and blood products in these countries, particularly in Ethiopia. Thus, identifying motivational factors affecting blood donation and recruitment of safe and low risk donors is necessary. For this reason, the study aimed at assessing knowledge, attitude, and practice towards blood donation and its associated factors. METHODS: A community based cross-sectional study was conducted in Debre Markos town from February to April, 2015...

BACKGROUND: Anticoagulant therapy is prescribed for millions of patients worldwide for the prevention and treatment of both arterial and venous thrombosis. Historically, only vitamin K antagonists have been available for clinicians to prescribe. The anticoagulation landscape is changing. The recent availability of the novel oral anticoagulants overcome many of the disadvantages associated with vitamin K antagonists. However the lack of formal monitoring and clinic follow-up is a concern for clinicians, as medication adherence is being assumed, which is known to decline in patients prescribed medications for chronic conditions...

BACKGROUND: Sickle cell disease (SCD) is endemic in non-Western countries. Due to migration, the prevalence of SCD in the Netherlands has increased. Adherence to medical treatment is recognized as a major problem area. Therefore, new effective interventions to increase adherence are urgently needed. METHODS/DESIGN: The TEAM study is an ongoing randomized controlled trial (RCT) to compare protocolized individual medical appointments (IMA's; care-as-usual) with protocolized group medical appointments (GMA's; novel intervention) in pediatric (n = 40) and adult (n = 60) patients...

BACKGROUND: Iron deficiency anemia is highly prevalent in patients with chronic kidney disease and is often treated with intravenous iron. There are few trials directly comparing the safety and efficacy of different intravenous iron products. METHODS: This post-hoc analysis pooled data from 767 patients enrolled in two randomized, controlled, open-label trials of similar design comparing the treatment of iron deficiency anemia with ferumoxytol and iron sucrose across patients with all stages of renal function...