Anaemia is the most common haematological disorder affecting humanity and is usually observed in chronic disease states such as non-specific anaemia, which may cause diagnostic difficulties. In chronically ill patients with anaemia, this has a negative impact on quality of life as well as survival. This paper aims at reviewing the pathogenesis of this form of anaemia with a view to suggesting future targets for therapeutic intervention. The ability to diagnose this disorder depends on the ability of the physician to correlate the possible clinical pathways of the underlying disease with the patients' ferrokinetic state. It is important to rule out iron deficiency and other causes of anaemia as misdiagnosis will in most cases lead to refractoriness to standard therapy. The cytokines and acute-phase proteins play important roles in the pathogenesis of anaemia of chronic disease. Alterations in the metabolism of iron via the molecule hepcidin and ferritin are largely responsible for the consequent anaemia. Concomitant iron deficiency might be present and could affect the diagnosis and therapeutic protocol. Treatment options involve the use of erythropoiesis-stimulating agents, blood transfusion, and iron supplementation, in addition to treating the underlying disease.

Background Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Nevertheless, their implementation in African countries has been mostly limited to pilot projects. Recent development of low-cost point-of-care testing (POCT) devices for sickle haemoglobin (HbS) could greatly facilitate the diagnosis of those affected. Methods We conducted the first multi-centre, real-world assessment of a low-cost POCT device, HemoTypeSC, in a low-income country. Between September and November 2017, we screened 1121 babies using both HemoTypeSC and HPLC and confirmed discordant samples by molecular diagnosis. Findings We found that, in optimal field conditions, the sensitivity and specificity of the test for SCA were 93.4% and 99.9%, respectively. All 14 carriers of haemoglobin C were successfully identified. Our study reveals an overall accuracy of 99.1%, but also highlights the importance of rigorous data collection, staff training and accurate confirmatory testing. It suggests that HPLC results might not be as reliable in a resource-poor setting as usually considered. Interpretation The use of such a POCT device can be scaled up and routinely used across multiple healthcare centres in sub-Saharan Africa, which would offer great potential for the identification and management of vast numbers of individuals affected by SCD who are currently undiagnosed. Funding US Imperial College London's Wellcome Trust Centre for Global Health Research (grant #WMNP P43370).

Background and study objectivesBlood transfusion is central in the prevention and treatment of certain chronic complications of sickle cell disease. It is indispensible in correcting anaemias as well as in the practice of exchange blood transfusion. These gains are largely limited by formation of allo-antibodies. Several studies demonstrated varying frequencies of allo-immunization in various patient groups. The effect of the racial differences between the donor and recipient pool, which has been subsumed in this study, has continuously created a confounding effect on the results of previous studies.AimThis study was aimed at determining the pattern and frequency of allo-immunization in multiply transfused sickle cell patients, in a racially matched donor and recipient population.Patients and methodsThis was a cross-sectional case-controlled study involving 80 Nigerian sickle cell disease patients who had received three or more units of packed red cells in the within 4 weeks of the study and 40 controls (who were SCD that had not been transfused in their life time). Antibody screening and identification was done using the Diamed microtyping system.ResultsFrequency of allo-immunization was determined to be 18.7 % (15/80) among the previously transfused and 5 % (15/120) in all sickle cell disease patients. Auto-antibodies were detected in 1.25 % of the study group and 2.5 % of the control, and all reacted with the Kell and Lutheran blood group antigens. The pattern of allo-antibodies found showed; 46.7 % Rhesus, 40 % Kell, while Lutheran and Duffy 13.3 %, each.ConclusionSickle cell disease patients are particularly susceptible to development of allo-antibodies despite racial similarities between the donor and recipient population. The most common allo-antibodies are Rhesus, Kell and Lutheran and Duffy respectively in order of decreasing frequency. Development of auto-antibodies seems to be independent of blood transfusion in sickle cell disease with possibly different pathogenetic mechanism. Policy on extended red cell phenotyping for common antigens will reduce allo-immunization among multiply transfused patients.

Background: Sickle cell disease, the inherited blood disorder characterized by anemia, severe pain and other vaso-occlusive complications, acute chest syndrome, disproportionate hospitalization, and early mortality, has significant financial, social, and psychosocial impacts and drains individuals, families, and health systems globally. Hydroxyurea could improve the health of the 300,000 individuals born each year with sickle cell disease in sub-Saharan Africa; however, challenges to adoption and adherence persist. This study assessed the barriers to therapeutic use of hydroxyurea for sickle cell disease within the Nigerian healthcare system, specifically from the level of the patient, provider, and health system.Methods: We used purposive sampling to recruit participants from 13 regions in Nigeria. A cross-sectional survey was administered to physicians (n = 70), nurses or counselors (n = 17), and patients or their caregivers (n = 33) at 13 health centers. Findings were mapped onto the appropriate Consolidated Framework for Implementation Research (CFIR) domains.Results: This study was able to identify factors that mapped onto the inner setting, outer setting, and characteristics of individuals domains of CFIR. The majority of physicians (74.3%) prescribe hydroxyurea, and half stated hydroxyurea is the standard of care. Among clinicians, barriers included limited knowledge of the drug, as well as low self-efficacy to prescribe among physicians and to counsel among nurses; perceived side effects; perceived patient preference for traditional medicine; cost for patient and expense of accompanying laboratory monitoring; and limited availability of the drug and equipment for laboratory monitoring. Among patients and caregivers, barriers included lack of knowledge; perceived side effects; cost; religious beliefs of disease causation; and lack of pediatric formulation.Conclusions: Findings suggest that patient, provider, and health systems-level interventions are needed to improve hydroxyurea uptake among providers and adherence among patients with sickle cell disease in Nigeria. Interventions such as patient education, provider training, and policy change could address the disproportionate burden of sickle cell disease in sub-Saharan Africa and thus improve health equity.

Objective: To evaluate the relationship between the occurrence of priapism and important steady-state clinical and laboratory parameters in homozygous sickle cell disease (SCD). Subjects and Methods: Steady-state clinical and laboratory data were obtained from the medical records of 126 male patients seen in the clinic over a 7-year period. Estimated prevalence rates, correlation coefficients and independent t tests were calculated to assess the relationship between priapism and several important clinical and laboratory indices. Patient data on age, haemoglobin concentrations, the frequency of crises per annum, leucocyte counts, platelet counts, serum bilirubin and aspartate transaminase were evaluated. Results: The prevalence of priapism was determined to be 21.4%, and 22.2% of those affected had erectile dysfunction. There was a significant positive correlation between priapism and older age (p = 0.049) and lower leucocyte counts (p = 0.008). There was no significant relationship with other clinical or laboratory indices. Conclusion: About 1 in 4 of all homozygous older SCD patients had priapism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.