Prevalence and type of monoclonal gammopathy of undetermined significance in an apparently healthy Nigerian population: a cross sectional study

Background The prevalence of monoclonal gammopathy of undetermined significance (MGUS), a premalignant plasma-cell disorder has not been determined in our geographic area Nigeria. Methods A cross sectional survey was carried on apparently healthy Nigerians selected by multistage sampling technique from the cosmopolitan city of Lagos, Nigeria. Subjects enrolled into the study had 2-step screening for the presence, type and concentration of monoclonal band. Agarose-gel electrophoresis was performed on all serum samples, and any serum sample with a discrete band of monoclonal protein or thought to have a localized band was subjected to Immunofixation. Subjects were also evaluated for Bence jones proteinuria, haematological and biochemical parameters. Results Four hundred and ten subjects with a mean age of 45.68 ± 10.3 years, a median of 45.00 years and a range of 20 to 80 years were enrolled into the study. MGUS was identified in only one (0.24 percent) of the 410 study subject. This subject was demonstrated to have a double monoclonal gammopathy; IgGλ at 16.9 g/L and IgAκ at 8.5 g/L. None of them including the sole subject with MGUS had a monoclonal urinary light chain. Conclusion Among residents of Lagos, Nigeria, MGUS was found in only 0.24% percent of apparently normal persons with a median age of 45 years. This suggests that MGUS which represents the earliest stage of monoclonal plasma/lymphoid cell proliferation is not a common finding in the relatively young population of Nigeria. Future epidemiologic studies dealing with plasma cell disorders in older people are required to carefully examine the relationship between environmental factors and prevalence of MGUS and its ultimate progression to MM

Cord blood full blood count parameters in Lagos, Nigeria

Introduction: Full blood count (FBC), one of the most frequently requested for laboratory investigations, is a simple, fast and cheap test and is a reliable indicator of health. Due to its usefulness in the  assessment of health status of individuals, its parameters in cord blood, a major source of haemopoietic stem cell transplantation and an ideal source for laboratory investigations for newborns were determined to provide a useful guide to local neonatologists and stem cell transplant physicians. Methods: Three millilitres of umbilical cord blood was collected from 130 normal birth weight newborns (69 males and 61 females) whose cord were clamped immediately after delivery, at a teaching hospital in Lagos, Nigeria and full blood count parameters were determined using Sysmex autoanalyzer, model  KX-21N. Consented mothers of the newborns were selected based on, age between 18 and 45 years;  uneventful pregnancy and delivery and haemoglobin (Hb) concentration ≥ 10 g/dL. Results: There were no statistical gender differences in the mean values of Hb concentrations (M=13.27  ±1.60 g/dL; F=13.32±1.61g/dL; p=0.93), total white cell count (M=3.16±5.43 × 109/L; F=13.07±4.98  × 109/L; p= 0.92), platelet count (M= 223.64± 64.21 × 109/L; F=226.69±80.83 × 109/L; p=0.81) and other parameters. Conclusion: Mean values of full blood count parameters obtained in this study are  comparable to reports from other studies in developing countries and could be a useful guide for neonatologists and stem cell transplant physicians in our geographical location.Key words: Haemoglobin, cord blood, stem cell, umbilical cord, neonatologis

Intravenous versus oral iron for iron deficiency anaemia in pregnant Nigerian women (IVON): study protocol for a randomised hybrid effectiveness-implementation trial.

BACKGROUND: Anaemia in pregnancy is highly prevalent in African countries. High-dose oral iron is the current recommended treatment for pregnancy-related iron deficiency anaemia (IDA) in Nigeria and other African countries. This oral regimen is often poorly tolerated and has several side effects. Parenteral iron preparations are now available for the treatment of IDA in pregnancy but not widely used in Africa. The IVON trial is investigating the comparative effectiveness and safety of intravenous ferric carboxymaltose versus oral ferrous sulphate standard-of-care for pregnancy-related IDA in Nigeria. We will also measure the implementation outcomes of acceptability, feasibility, fidelity, and cost-effectiveness for intravenous ferric carboxymaltose. METHODS: This is an open-label randomised controlled trial with a hybrid type 1 effectiveness-implementation design, conducted at 10 health facilities in Kano (Northern) and Lagos (Southern) states in Nigeria. A total of 1056 pregnant women at 20-32 weeks' gestational age with moderate or severe anaemia (Hb < 10g/dl) will be randomised 1:1 into two groups. The interventional treatment is one 1000-mg dose of intravenous ferric carboxymaltose at enrolment; the control treatment is thrice daily oral ferrous sulphate (195 mg elemental iron daily), from enrolment till 6 weeks postpartum. Primary outcome measures are (1) the prevalence of maternal anaemia at 36 weeks and (2) infant preterm birth (<37 weeks' gestation) and will be analysed by intention-to-treat. Maternal full blood count and iron panel will be assayed at 4 weeks post-enrolment, 36 weeks' gestation, delivery, and 6 weeks postpartum. Implementation outcomes of acceptability, feasibility, fidelity, and cost will be assessed with structured questionnaires, key informant interviews, and focus group discussions. DISCUSSION: The IVON trial could provide both effectiveness and implementation evidence to guide policy for integration and uptake of intravenous iron for treating anaemia in pregnancy in Nigeria and similar resource-limited, high-burden settings. If found effective, further studies exploring different intravenous iron doses are planned. TRIAL REGISTRATION: ISRCTN registry ISRCTN63484804 . Registered on 10 December 2020 Clinicaltrials.gov NCT04976179 . Registered on 26 July 2021 The current protocol version is version 2.1 (080/080/2021)

Intravenous versus oral iron for iron deficiency anaemia in pregnant Nigerian women (IVON): study protocol for a randomised hybrid effectiveness-implementation trial

Background Anaemia in pregnancy is highly prevalent in African countries. High-dose oral iron is the current recommended treatment for pregnancy-related iron deficiency anaemia (IDA) in Nigeria and other African countries. This oral regimen is often poorly tolerated and has several side effects. Parenteral iron preparations are now available for the treatment of IDA in pregnancy but not widely used in Africa. The IVON trial is investigating the comparative effectiveness and safety of intravenous ferric carboxymaltose versus oral ferrous sulphate standard-of-care for pregnancy-related IDA in Nigeria. We will also measure the implementation outcomes of acceptability, feasibility, fidelity, and cost-effectiveness for intravenous ferric carboxymaltose. Methods This is an open-label randomised controlled trial with a hybrid type 1 effectiveness-implementation design, conducted at 10 health facilities in Kano (Northern) and Lagos (Southern) states in Nigeria. A total of 1056 pregnant women at 20–32 weeks’ gestational age with moderate or severe anaemia (Hb < 10g/dl) will be randomised 1:1 into two groups. The interventional treatment is one 1000-mg dose of intravenous ferric carboxymaltose at enrolment; the control treatment is thrice daily oral ferrous sulphate (195 mg elemental iron daily), from enrolment till 6 weeks postpartum. Primary outcome measures are (1) the prevalence of maternal anaemia at 36 weeks and (2) infant preterm birth (<37 weeks’ gestation) and will be analysed by intention-to-treat. Maternal full blood count and iron panel will be assayed at 4 weeks post-enrolment, 36 weeks’ gestation, delivery, and 6 weeks postpartum. Implementation outcomes of acceptability, feasibility, fidelity, and cost will be assessed with structured questionnaires, key informant interviews, and focus group discussions. Discussion The IVON trial could provide both effectiveness and implementation evidence to guide policy for integration and uptake of intravenous iron for treating anaemia in pregnancy in Nigeria and similar resource-limited, high-burden settings. If found effective, further studies exploring different intravenous iron doses are planned. Trial registration ISRCTN registry ISRCTN63484804. Registered on 10 December 2020. Clinicaltrials.govNCT04976179. Registered on 26 July 2021 The current protocol version is version 2.1 (080/080/2021)

Serum testosterone levels of HbSS (sickle cell disease) male subjects in Lagos, Nigeria

<p>Abstract</p> <p>Background</p> <p>Infertility is a major problem in sickle cell disease patients, especially in males. In addition to low serum testosterone, other abnormalities involving the accessory sex organs, such as the seminal vesicles and the prostate gland, as well as marked decrease in ejaculate volume may be observed in male HbSS patients. Hence, the need to study the role of sex hormones as a cause of infertility in male HbSS patients.</p> <p>Methods</p> <p>An unmatched case-control study was performed using seventy-five consenting subjects from Lagos University Teaching Hospital. These included 47 patients with haemoglobin phenotype SS from the Sickle cell clinic and 28 volunteered medical students and members of staff with haemoglobin phenotype AA. Demographic data were obtained using a self-administered questionnaire. A total of 5 mls of blood was collected from each subject between 9.00 am & 11.am, and assayed for serum testosterone concentration.</p> <p>Results</p> <p>The concentrations of serum testosterone in HbSS patients ranged from 0.2 to 4.3 ng/ml with a mean of 1.28 ± 0.72 ng/ml whilst the values in HbAA controls ranged from 1.2 to 6.9 ng/ml with a mean of 2.63 ± 1.04 ng/ml. Seven (25.0%) of the 28 controls had serum testosterone concentration lower than the quoted reference (normal) range whereas 44 (93.6%) of the 47 HbSS subjects had serum testosterone concentration lower than the reference range.</p> <p>Conclusion</p> <p>Overall, subjects with HbSS have significantly lower mean serum testosterone than HbAA controls.</p

Utilisation of SF-36 and WHOQOL-BREF in assessing health related quality of life of people living with sickle cell disease in Nigeria

Background: Health-related quality of life (HRQoL) can be assessed with generic or disease-specific HRQoL instruments. The use of a generic tool will however allow comparison between Sickle Cell Disease (SCD) with other populations. To provide accurate data on HRQoL however, the instrument must be a valid tool to measure this outcome.Objective: The study is to determine and compare the utility of two generic HRQoL tools, SF-36 and WHOQOLBREF in assessing HRQoL in Nigerians with SCD to determine which is better suited for the population.Methods: The study was a cross-sectional survey of 180 young adult with SCD. HRQoL was evaluated using the Short Form 36 version 2 Health Survey (SF-36v2) and WHOQOL-BREF. Internal consistency, reliability and sensitivity were determined and compared. The degree of correlation between both tools in measuring HRQoL in the same population was also determined.Results: SF-36 showed better internal consistency (0.61- 0.90) than the WHOQOL-BREF (0.32-0.90), with the former less affected by ceiling and floor effects. Both instruments were equally sensitive to discriminate between participants with SCD related complication and recent hospitalisation from those without SCD related complication and recent hospitalisation with those with complications/hospitalisation reporting significantly lower HRQoL scores with both instruments. Both also showed weak to strong positive correlations in their measures.Conclusion: The SF-36 and WHOQOL-BREF are both useful tools in assessing HRQoL in people with SCD in Nigeria. Whilst they overlap in measuring certain segments of HRQoL; they show strength for different domains of HRQoL.Keywords: Sickle cell disease, Quality of life, SF-36, WHOQOL BREF, Nigeri

Orofacial manifestation of hematological disorders: Hemato-oncologic and immuno-deficiency disorders

The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases with special reference to hemato-oncologic, immuno-deficiency disorders, and human immunodeficiency virus infection. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases with emphasis on hemato-oncologic and human immunodeficiency virus (HIV) infection. Mesh phrases used in the search were: Oral diseases AND hematological disorders; orofacial diseases AND leukemias; orofacial lesions AND lymphomas; orofacial diseases AND multiple myeloma, orofacial manifestations AND HIV. The Boolean operator "AND" was used to combine and narrow the searches. The full texts of these articles were thoroughly examined. References in these articles also were manually searched non-Medline articles. Only relevant articles were selected for the review. Orofacial manifestation of malignant hematological diseases may present as primary clinical features due to infiltration of orofacial tissues, or as secondary due to the subsequent infiltration of normal bone marrow elements, or tertiary due to the side effects of the treatment. HIV-associated orofacial lesion may be a clinical indicator of HIV infection in otherwise healthy, undiagnosed individuals; an early clinical feature of HIV infection; clinical markers for the classification and staging of HIV disease or may be a predictor of HIV disease progression. Orofacial manifestations of malignant hematological diseases and HIV infection are not uncommon findings in clinical practice. These manifestations may be clinical indicators of hematologic disorders in otherwise healthy, undiagnosed individuals

The 8p12 myeloproliferative syndrome

The occurrence of a myeloproliferative disorder in association with an aggressive lymphoproliferative disorder is a distinctly unusual phenomenon. We report a case of concurrent leukaemia-lymphoma syndrome characterized by a BCR/ABL-negative myeloproliferative disease, eosinophilia and a lymphoma. The bone marrow chromosome analysis showed the karyotype 46, XY, t(8;9) (q12; p33), which indicated presence of FGFR1 gene translocations. 8p12 myeloproliferative syndrome (EMS) / stem cell leukaemia-lymphoma syndrome (SCLL) belongs to the tyrosine kinase fusion genes chronic myeloproliferative diseases. The patient was managed conservatively with hydroxyurea, allopurinol and blood component therapy. The patient eventually died of intracerebral haemorrhage due to severe thrombocytopaenia. Based on our experience the overlap in the clinical presentation of this disease with lymphomas, can lead to a delay in diagnosis of EMS/SCLL. Given the aggressive nature of this disease, an accurate clinical and molecular diagnosis of this entity has become increasingly important

Sickle cell disease: caregiver’s awareness and phenotype distribution among children presenting to children emergency of a tertiary hospital in Lagos, Nigeria

Background: Sickle cell disease accounts for significant morbidity and mortality in sub-Saharan Africa and the burden is expected to increase further by 2050. Nigeria is known to bear the highest burden of sickle cell disease in the world with about 2.69–5% of the population affected by the disease.Aim: This study determined awareness of sickle cell disease among caregivers and phenotype distribution of children presenting to children emergency in Lagos University Teaching Hospital (LUTH), Nigeria.Methods: The study was crosssectional and descriptive in design and data was collected using a pretested, structured intervieweradministered questionnaire among 250 caregivers and children. HemoTypeSC™ rapid test kit was used to determine the hemoglobin&nbsp; phenotype in whole blood of the respondents who were consecutively recruited following the caregiver’s consent. The Statistical Package for Social Sciences version 21 software was used for analysis.Univariate and bivariate analyses were carried out with a level of significance set at p ≤ 0.05.Results: The mean age of the children was 50.27±50.91 months. There were more females 141 (56.4%) than males. Almost all 242 (96.8%) caregivers did not know the children’s Hb phenotype. Most 173 (69.2%) of the children had HbAA;55(22.0%) were HbAS; 6(2.4%) were HbAC; 15 and 1 (6.0% and 0.4%) were HbSS and HbSCphenotypes respectively. Education was statistically significant with awareness of SCD (p=0.002) and awareness of SCD was statistically significant with knowledge of prevention (p&lt;0.001) among the caregivers.Conclusion: Awareness of SCD among the caregivers of children was high, although the majority of them did not know the children’s Hb phenotype. Most of the children had HbAA with a high proportion of HbSS and HbSC phenotypes. A routine neonatal/early infant screening program for SCD is highly recommended in Nigeria for early diagnosis and prevention of SCD complications