Prevalence of venous thromboembolism and its associations in a large racially homogenous population of sickle cell disease patients.

To determine the prevalence of venous thromboembolism (VTE) among adult sickle cell disease (SCD) patients in Nigeria. METHODS: This was a multicentre retrospective study in which the medical records of adult SCD patients were reviewed. Information on demographics, steady-state haemogram, clinical phenotypes, duration of follow-up, history of VTE including risk factors and management was collected. RESULTS: Of the 509 SCD patients with a median (IQR) duration of follow-up of 2 years, 10 (2.0%) had VTE (9 DVT and 1 PE). Their median (IQR) age was 27 (22.8-30.3) years. Identifiable risk factors for VTE included positive family history (2, 20%) surgery, splenectomy, paraplegia and cancer (1, 10% each). No risk factor was identifiable in four persons. VTE had no significant association with age and gender. VTE was significantly associated with the following events: acute chest syndrome [p = .002, odds ratio (OR) 8, 95% CI 2.2-28.9], osteonecrosis [p = .012, OR 5.24, 95% CI, 1.45-18.91] and vaso-occlusive crisis [p = .035]. Also significantly associated with VTE were pulmonary hypertension [p = .001, OR 23.3, 95%CI 5.18-105.06] and stroke [p = .032, OR 9.35, 95%CI 0.87-53.25]. CONCLUSION: The prevalence of VTE among SCD patients in Nigeria is low. It is significantly associated with vaso-occlusive crisis, pulmonary hypertension and stroke.

Blood donation practices, processing and utilisation of blood components in government tertiary hospitals in Nigeria: a multicentre cooperative study.

BACKGROUND: Timely access to safe blood and blood components is still a challenge in Nigeria. This study aimed to determine blood donation practices, processing and utilization of blood components across government tertiary hospitals (THs) in Nigeria. METHODS: This was a descriptive cross-sectional study done in Nigeria in June-July 2020. Data were analysed with SPSS version 21.0. RESULTS: Data were collected from 50 THs. The majority (68%) of the THs lack facilities for blood component preparation and only 18% and 32% provide cryoprecipitate and platelet concentrate, respectively. Whole blood was most commonly requested (57.04%). All facilities tested blood for HIV, HBV and HCV, but the majority (23 [46%]) employed rapid screening tests alone and nucleic acid testing was not available in any hospitals. The manual method was the most common method of compatibility testing in 90% (45/50) and none of the THs routinely perform extended red cell typing. The average time to process routine, emergency and uncross-matched requests were a mean of 109.58±79.76 min (range 45.00-360.00), 41.62±25.23 (10.00-240.00) and 11.09±4.92 (2.00-20.00), respectively. CONCLUSION: Facilities for blood component preparation were not widely available. Concerned government authorities should provide facilities for blood component preparation.

Plasminogen Activator Inhibitor 1 (PAI-1) and Haematologic Parameters in HIV- Positive Patients on Protease Inhibitor-Based HAART Regimen: A Case-Control Study.

Background: Human immunodeficiency virus (HIV) infection is associated with an increased risk of hypercoagulability and treatment with antiretroviral agents especially protease inhibitors has also been reported to contribute to this risk. Altered fibrinolytic activity is reported as a mechanism of increased thrombotic risk in HIV patients on therapy. However, this has not been investigated in our environment. Objective: To evaluate and compare PAI-1 levels as a marker of thrombotic risk in HIV-infected persons on PI-based HAART regimen with those on non-PI-based therapy and to correlate PAI-1 with haematological parameters. Methods: This was a comparative cross-sectional study conducted at the University of Calabar Teaching Hospital (UCTH), Calabar, Cross River State. A total of 125 subjects including 45 HIV-positive patients on PI-based HAART regimen, 42 HIV-positive patients on non-PI-based HAART regimen and 38 Controls. The controls include 18 HIV-positive therapy naïve patients and 20 HIV-negative controls. PAI 1 and blood counts were estimated using standard methods. Data were analyzed using the IBM version of the statistical package for social sciences (SPSS) version 22. Statistical significance was set at 0.05. Result: The median PAI-1 level was significantly increased in patients on PI-based HAART regimen (p = 0.004). The blood counts did not differ significantly between patients on PI and non-PI-based HAART regimens (p > 0.05). There were no significant correlations between PAI-1 levels and blood counts (p > 0.05). Conclusion: PAI-1 level is elevated in HIV patients on PI-based HAART regimen. However, the association with thrombotic events could not be established in the study.

Determination of von Willebrand factor level in patient with sickle cell diseasein vaso-occlusive crisis.

BACKGROUND: Sickle cell anemia (SCA) is a hypercoagulable state characterized by a significant alteration in hemostatic parameters that may predispose to increased risk of vaso-occlusive crisis (VOC). The role of von Willebrand factor (VWF) in the pathogenesis of VOC has not been fully investigated in Nigeria. OBJECTIVE: The objective of this study was to evaluate the level of VWF in subjects with sickle cell disease (SCD) in Calabar, Nigeria, to determine its role in the pathogenesis of VOC. METHODS: This was a comparative study carried out at the University of Calabar Teaching Hospital, Calabar, Nigeria. Sixty patients with SCA in VOC and 50 healthy controls were included. VWF levels were measured using Assaypro enzyme-linked immunosorbent assay kits. RESULTS: The mean age of patients with SCA in VOC and controls was 23.5 ± 7.2 years and 26.5 ± 5.6 years, respectively. The means (standard deviations) of VWF in patients in VOC and controls were 2.52 ± 0.34 IU/mL and 1.41 ± 0.23 IU/mL, respectively. There was no correlation of hematocrit and VWF in VOC (r = -0.034; P = .80), while there was a modest inverse correlation in controls. CONCLUSIONS: Levels of VWFare elevated in a VOC state and thus may be implicated in the pathogenesis of VOC.

Health Status of Persons with Hemophilia: A Pilot Survey from a Resource-Constrained Country.

BACKGROUND: Most resource-poor countries are yet to develop standard hemophilia treatment center (HTC) despite improved outcome of health status of persons with hemophilia (PWH). AIM: This study aimed to evaluate the health status of PWH in Nigeria. METHODOLOGY: In this descriptive, cross-sectional study, modified prevalidated and pretested questionnaire (National health and Nutrition examination survey (NHANES) 2013 Health Status questionnaire (HSQ)) was consecutively administered to consenting PWH (pediatric and adult) attending the 2018 Annual General Meeting of the Haemophilia Foundation of Nigeria. The study's measurable outcome variables were calculated health status and its determinants. Association between the outcome variables and clinical characteristics of PWH was done using SPSS software version 22, and P < 0.05 was considered statistically significant. RESULTS: Of the 36 PWH who participated in the survey, 50% had good health status, 38.9% had poor health status, while only 11.1% had excellent health status. A majority (88.9%) had access to HTC with <6 consultations in the past year. Nearly 47.2% were hospitalized for disease-related problem in the past year. There was nonsignificant difference between health status and disease type (P = 0.751) and severity (P = 0.086), treatment plan (0.496), type of treatment facility (P = 0.152), and access to a doctor (P = 0.67). CONCLUSION: Several PWH in resource-poor settings still suffer serious morbidity that impacts negatively on their health status. More robust (multicenter) research is needed to ascertain the true picture of health status of PWH in resource-poor countries.

Bortezomib Prescription Pattern for the Treatment of Multiple Myeloma by Hematologists in Nigeria.

PURPOSE: Novel therapy has dramatically changed the outcome of patients with myeloma. Current National Comprehensive Cancer Network guidelines give bortezomib-based combinations a central role in the management of multiple myeloma (MM). The aim of this survey is to assess the use of bortezomib for the treatment of MM by hematologists practicing in Nigeria. MATERIALS AND METHODS: This is a cross-sectional observational survey. A structured, prevalidated questionnaire was self-administered to different cadres of hematologists. Data collected were analyzed using SPSS software version 21 (IBM, Chicago, IL). RESULTS: There were 54 respondents from 24 centers across the country. The most frequently used drugs for first-line therapy were thalidomide (66.7%), dexamethasone (54.2%), and bortezomib (48%), and a combination of bortezomib, thalidomide, and dexamethasone (16.7%) was the most frequently used first-line regimen. Of the 54 hematologists, 39 (72.2%) had prescribed bortezomib previously; no one had used bortezomib as monotherapy. Drug unavailability (86.7%) and cost (46.7%) were the major reasons for those who had not prescribed bortezomib. Approximately 56.4% of responders had patients who had experienced adverse effects, of which neuropathy was the most common (86.3%). CONCLUSION: Bortezomib, thalidomide, and dexamethasone was the most frequently used first-line regimen to treat myelomatosis. Thalidomide and dexamethasone were the most frequently used drugs in myeloma treatment. Despite poor access to health care, coupled with the high cost and poor availability of bortezomib in our low- or middle-income country, those who prescribed bortezomib did so frequently (in more than half of their patients).

Correlation of hematocrit and Apgar scores in newborns of women with hypertensive disorders in pregnancy.

OBJECTIVES: To compare the incidence of polycythemia in newborns of women with hypertensive disorders in pregnancy (HDP) with those of normotensive mothers, to determine the incidence of perinatal stress using Apgar scores and to correlate hematocrit with Apgar scores in these newborn. STUDY DESIGN: This was a hospital-based comparative study conducted in the University of Port Harcourt Teaching Hospital, Nigeria. MAIN OUTCOME MEASURES: Apgar scores of 200 newborns- 100 from mothers with HDP (case group) and 100 from normotensive mothers (control group)- were taken at 1st and 5th minute of birth and cord blood samples collected to determine hematocrit. The subjects were categorized into polycythemic and non polycythemic using a hematocrit ≥65%. RESULTS: Eight percent of newborns of women with HDP had polycythemia while none of the controls did. Apgar scores in the case group with and without polycythemia at one-minute were 4.1±1.8 and 6.6±2.1, respectively and at 5 minutes were 6.9±1.7 and 8.5±1.4 respectively. Hematocrit correlated positively with Apgar scores (both at one and five minutes) in cases without polycythemia (r = 0.221, p = 0.034 and r = 0.255, p = 0.014). Hematocrit of polycythemic newborns did not correlate with Apgar scores (r = -0.287, p = 0.491 and r = -0.436, p = 0.281). CONCLUSION: The incidence of polycythemia is significantly higher in newborns of women with HDP and these polycythemic neonates had a significantly higher incidence of birth asphyxia. Therefore, birth outcome as determined by Apgar score is influenced by hematocrit.

First successful allogeneic hematopoietic stem cell transplantation for a sickle cell disease patient in a low resource country (Nigeria): a case report.

BACKGROUND: Sickle cell disease (SCD) has a prevalence of 2-3% in Nigeria (population: over 150 million). We present our first allogeneic hematopoietic stem cell transplantation (HSCT) for a 7-year-old patient with severe sickle cell anemia and debilitating right-sided hemi-paraparesis. CASE REPORT: Conditioning was with (Reduced Intensity Conditioning (FLU/BU).[Fludarabine 160 mg/m2 (days -6 to -2) and Busulphan 16 mg/kg (4×25 mg 6 hly days -5 to -2) and Anti-thymocyte globulin(ATG)(ATGAM) total dose 500 mg (days -6 to -4)]. Graft versus Host Disease (GVHD) prophylaxis was with Cyclosporine A (2×50 mg daily) and Mycophenolate Mofetil (2×500 mg/day). Stem cell source was bone marrow harvested on the 28 September 2011 with 9.8×108 nucleated cells/kg in a total volume of 900 mL from his 14-year-old HLA-matched sibling (6/6). Neutrophil and platelet engraftment was day +18 and +21, respectively. At day +70 full blood count was a total white blood cell count of 3100/µl, absolute Neutrophil count 1200/µl, Hemoglobin (Hb) 11.3 g/dl, Platelet 198,000/µl, Hemoglobin phenotype AA, and no acute or chronic GVHD. He is clinically stable with a Chimerism at 2 years post-HSCT of 95% and responding to physiotherapy. CONCLUSIONS: We have successfully performed a stem cell transplanted in a 7-year-old Sickle Cell Anemia case. With the assistance of Government and improved Health Insurance Policy, we could make HSCT available as a cure for many Nigerians with both malignant and non-malignant disorders.

Knowledge, Attitude, and Practice of Voluntary Blood Donation among Healthcare Workers at the University of Benin Teaching Hospital, Benin City, Nigeria.

Introduction. Adequate and safe blood supply has remained a challenge in developing countries like ours. There is a high dependency on family replacement and remunerated blood donors in our environment which carries an attendant increased risk of transfusion transmissible infection. Objectives. The objectives of this study were to assess the knowledge, attitude, and practice of voluntary blood donation among healthcare workers (nonphysicians) and to identify and recruit potential voluntary blood donors. Methodology. This was a cross-sectional descriptive study carried out at the University of Benin Teaching Hospital, Benin City. A total of 163 staffs were recruited. Pretest questionnaires were used to assess their knowledge, attitude, and practice of voluntary blood donation. Statistical Analysis. The responses were collated and analyzed with the Statistical Package for Social Sciences (SPSS) 16. The association between blood donation practice and gender of respondents, category of staff, and level of education was tested using Chi-square and Fisher's tests where appropriate. P < 0.05 were considered statistically significant. Results. The median age of the respondents was 32 years (18-56) with females accounting for 55.6% (90). A total of 74.8% (122) attained tertiary education, and 55.8% (91) of respondents were senior staffs. The majority has good knowledge and positive attitude towards donation; however, only 22.1% (36) have donated blood with 41.7% (15) of these being voluntary. Male workers were more likely to donate (P < 0.05). There is no significant association between blood donation and level of education. Conclusion. There is a strong disparity between the knowledge, attitude, and practice of voluntary donation amongst healthcare workers.

Donor blood procurement and the risk of transfusion transmissible viral infections in a tertiary health facility in South-South Nigeria.

BACKGROUND: Blood and blood products are scarce commodities. The demand often outweighs the supply. This study is directed at investigating the blood procurement sources and the risk of viral transfusion transmissible infection. MATERIALS AND METHODS: The records of the blood transfusion unit of a tertiary health facility in south-south Nigeria were studied. The procurement and screening records from 1 January to 31 December 2009 were analyzed. RESULTS: 7,552 donor records were analyzed, 6,931 were commercial donor and 621 replacement donors. 891 commercial donors were infected, 500 (7.2%) were HIV positive, 323 (4.7%) HBV positive, 42 (0.6%) had HIV and HBV co-infection, while 28 (0.4%) were HCV positive. Twenty-three replacement donors were infected, 16 (2.6%) were HIV positive, 6 (1%) were HBV positive, while 1 (0.2%) were HCV positive. None of the replacement donors had co-infection. The risk of infection was significantly higher with commercial donor procurement (X2=45.07, P<0.001, OD=3.845). CONCLUSION: Commercial blood donors are still the major source of blood to the hospital and they also have the highest prevalence of transfusion transmissible viral infections in this region thus constitute a major risk transmitting infections to potential recipients.