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.

Perception to hydroxyurea therapy in patients with sickle cell disease: Report from 3 centers.

Background: Hydroxyurea (HU) is an hemoglobin F inducing agent used in the treatment of sickle cell disease (SCD). Aim: The aim of this study is to determine the perception of HU by people living with SCD. Materials and Methods: A pretested questionnaire was self-administered to known cases of SCD attending pediatrics and adult hematology clinics in three participating centers. Mothers of children <18 years responded on their behalf. Results: There were 101 responders, 49 (48.5%) males and 52 (51.5%) females, of which 24 (23.8%) were children <18 years and 77 (76.2%) were adults. The majority (n = 73, 72.3%) knew their phenotype. Up to 63 (62.4%) had crises in the past 3 months. Only 35 (34.7%) had heard of HU, many through their doctor (n = 16, 45.7%), 8 (22.9%) through online resources, and 7 (20%) from friends. Only 12 (11.9%) had been exposed to HU therapy, of which 5 (41.7%) had discontinued therapy mostly due to side effects (n = 2, 40%). The seven patients (58.3%) on continuous HU therapy for a duration of 6 months to over 5 years, all reported reduced hospital admissions and frequency of crises as benefits of the drug, whereas 4 (57.1%) had stopped requiring blood transfusion since starting therapy. Of those who had never taken HU, 53 (52.5%) believed that HU should be used in treating SCD and majority (n = 32, 60.4%) would want to be commenced on the drug. However, 8 (15.1%) would decline therapy (mostly due to perceived associated side effects; n = 4; 50%). Six (11.3%) were unsure if they would want the drug and 7 (13.2%) would have to discuss the decision first with their family. There were 8 (8.9%) responders who did not think HU will be beneficial in SCD and would decline treatment, while 26 (29.2%) were unsure of both the benefits of the drug or of commencing therapy. Conclusion: The findings from this study suggest that HU is beneficial for patients with SCD; however, the awareness of this medication among SCD patients is still low in our environment. Some SCD patients would decline the use of HU due to perceived side effects. We recommend that more awareness on HU be created and coordinated multi-center studies on the efficacy of HU in the Nigerian population be carried out.

RésuméContexte: L'hydroxyurée (HU) est un agent inducteur de l'hémoglobine F utilisé dans le traitement de la drépanocytose (SCD). Objectif: le but de cette L'étude vise à déterminer la perception de l'HU par les personnes atteintes de SCD. Matériel et Méthodes: un questionnaire pré-testé a été auto-administré aux cas connus de SCD fréquentant des cliniques de pédiatrie et d'hématologie pour adultes dans trois centres participants. Les mères d'enfants de moins de 18 ans ont répondu en leur nom. Résultats: Il y avait 101 répondants, 49 (48,5%) hommes et 52 (51,5%) femmes, dont 24 (23,8%) étaient des enfants de moins de 18 ans et 77 (76,2%) étaient des adultes. La majorité (n = 73, 72,3%) connaissait leur phénotype. Jusqu'à 63 (62,4%) ont eu des crises au cours des 3 derniers mois. Seulement 35 (34,7%) avaient entendu parler de HU, beaucoup par l'intermédiaire de leur médecin (n = 16, 45,7%), 8 (22,9%) par des ressources en ligne et 7 (20%) par des amis. Seulement 12 (11,9%) avaient été exposés à un traitement par HU, dont 5 (41,7%) avaient arrêté le traitement principalement en raison d'effets secondaires (n = 2, 40%). Les sept patients (58,3%) sous traitement HU continu pendant une durée de 6 mois à plus de 5 ans, tous ont signalé une réduction des hospitalisations et de la fréquence des crises comme bienfaits du médicament, alors que 4 (57,1%) avaient cessé de nécessiter une transfusion sanguine depuis le début du traitement. De ceux qui n'avaient jamais prises HU, 53 (52,5%) estimaient que HU devrait être utilisée dans le traitement de la drépanocytose et la majorité (n = 32, 60,4%) souhaiterait commencer le médicament. Cependant, 8 (15,1%) refuseraient le traitement (principalement en raison des effets secondaires associés perçus; n = 4; 50%). Six (11,3%) ne savaient pas siils voudraient le médicament et 7 (13,2%) devraient d'abord discuter de la décision avec leur famille. Il y avait 8 répondants (8,9%) quine pense pas que l'HU sera bénéfique dans la drépanocytose et refuserait le traitement, tandis que 26 (29,2%) n'étaient pas certains des avantages du médicament ou commencer la thérapie. Conclusion: Les résultats de cette étude suggèrent que l'HU est bénéfique pour les patients atteints de SCD; cependant, la conscience de ce médicament chez les patients SCD est encore faible dans notre environnement. Certains patients SCD refuseraient l'utilisation de l'HU en raison du côté perçu effets. Nous recommandons qu'une plus grande sensibilisation à l'HU soit créée et coordonne des études multicentriques sur l'efficacité de l'HU au Nigéria. population être effectuée.

Chronic Lymphocytic Leukemia: Prognostic Factors at Presentation in a Resource-Limited Center.

PURPOSE: Determining chronic lymphocytic leukemia (CLL) prognosis using the International Prognostic Index markers such as TP53 and immunoglobulin heavy-chain variable region gene mutation in a resource-limited setting is difficult to achieve because of cost and equipment unavailability. The aim of this study is to determine prognostic factors easily available to hematologists in low- or medium-income countries. MATERIALS AND METHODS: This was a retrospective study conducted at the University of Port Harcourt Teaching Hospital, Nigeria. Data were retrieved from CLL patient records from January 2004 to December 2019 (15 years). Data collected were analyzed using SPSS software version 25. RESULTS: A total of 46 records were reviewed, with a median age of 55 years and a male:female ratio of 1:1.2. All patients were symptomatic at presentation, with splenomegaly (91.3%), anemia (82.6%), and lymphadenopathy (76.1%) predominating. About 89.1% of the patients presented at Binet stage C and/or high-risk Rai (Rai stages III and IV) with 10.9% presenting at Binet stage B and/or intermediate-risk Rai (Rai stage II). Only 13% of the patients had immunophenotyping done with 6.5% being done for the Matutes CLL score. The 5-year overall survival (OS) was 15.7% with a median survival of 26 months. WBC count and absolute lymphocyte count (ALC) > 100 × 109/L were significant poor prognostic markers (P = .013 and .021, respectively). Thirty-five (76.1%) received chemotherapy, and they had a better median survival than those who did not (26 v 17.5 months). The most common regimen used was cyclophosphamide, vincristine, and prednisolone for 15 (42.9%) patients. CONCLUSION: WBC count and ALC > 100 × 109/L were poor prognostic markers. Patients who received chemotherapy had a better OS.

Presenting features and treatment outcomes of chronic lymphocytic leukaemia in a resource poor Southern Nigeria.

Background: Chronic lymphocytic leukaemia is a relatively common haematological malignancy affecting older adults, accounting for about 20% of haematological malignancies in Nigeria. Diagnosis of this disease depends on the demonstration of clonal lymphocytosis > 5 × 109/L with a characteristic immunophenotypic pattern amidst other clinical and laboratory features. Objectives: To determine the predominant clinical and laboratory features of CLL at presentation and their relationship with patient survival. This study also aims at examining the relationship between treatment protocol and outcome. Methods: This is a retrospective study with 8 years data (2010-2018) collected from four different centers. Data was analyzed using SPSS 20.0. Results: There were a total of 97 cases, with a male: female ratio of 1.1:1. The median age at presentation was 59 years. Approximately 55% of the patients presented at Binet stage C, with splenomegaly in 93.2% and 78% were anaemic. The mean white cell count was 137.9 ± 14.7 × 109/L, with a median absolute lymphocyte count of 86 × 109/L. The commonest treatment regimen was chlorambucil and prednisolone and males had a superior response. The number of chemotherapy cycles, serum alkaline phosphatase and aspartate transaminase correlated positively with duration of survival. Mortality rate over the five year period was 14.3%. Conclusion: CLL was found to present in younger patients when compared to previous studies with a median age of 57 years at diagnosis. Our study showed a slight female preponderance and better response to therapy in males. Majority of the patients presented in Binet stage C and were treated with chlorambucil-based drug combinations compared to more current treatment with Fludarabine-based combinations. A high serum alanine transaminase and alkaline phosphatase was found to positively correlate with survival amongst this patient population.

Correlates of neutrophil/lymphocyte, platelet/lymphocyte, and platelet/neutrophil ratios of neonates of women with hypertensive disease of pregnancy with neonatal birth outcomes.

BACKGROUND: Studies have indicated neutrophil/lymphocyte ratio (NLR), Platelet/Lymphocyte ratio (PLR), and platelet/neutrophils ratio (PNR) to be inflammatory markers. The correlation of these values in infants of hypertensive mothers has not been investigated. AIM: To investigate the relationship between NLR, PLR, and PNR of neonates of women with hypertensive disease of pregnancy and neonatal APGAR scores and birth weight. METHODOLOGY: Cord blood samples of 200 neonates collected and the blood counts and ratios obtained. RESULTS: NLR in the babies of the hypertensive mothers was 0.865 and 1.42 in the control group (p = 0.0001). PLR was 34.7 in the neonates of the hypertensive mothers and 62.4 in the control group (p = 0.0001). PNR did not differ significantly between the two groups,p = 0.418. Degree of hypertension had a direct relationship with NLR; SBP had a p value of 0.001 while the DBP had p = 0.002. The PLR had an inverse relationship with the degree of hypertension; SBP p value of 0.0001, while DBP was p = 0.0001. No significant association was observed between the ratios and neonatal birth weight (p ≥ 0.05); however, PNR and PLR were found to be significantly associated with the 1st (p = 0.045 and 0.030) and 5th (0.049 and 0.037) minute APGAR scores in the newborns. CONCLUSION: PLR and NLR in neonates of hypertensive mothers are found to be markedly lower than those of controls, the degree of which is affected by the severity of hypertension. Also, lower PLR is associated with lower APGAR scores. Therefore, severity of high blood pressure and lower PLR may be determinants of poor birth outcome.

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).

Management of aplastic anaemia in pregnancy in a resource poor centre.

Aplastic anaemia occurring in pregnancy is a rare event with life threatening challenges for both mother and child. We present a successful fetomaternal outcome despite the challenges in the management of this rare condition in a tertiary but resource poor centre. This is case of a 37 year old Nigerian woman G6P0+5managed with repeated blood transfusions from 28 weeks of gestation for bone marrow biopsy confirmed aplastic anaemia following presentation with weakness and gingival bleeds. She had a cesarean section at 37 weeks for pre-eclampsia and oligohydraminous with good feto-maternal outcome. She was managed entirely with fresh whole blood and received 21 units. Aplastic Anaemia in Pregnancy is a rare event with poor feto maternal prognosis. Successful management is possible with good multi-disciplinary approach and availability of supportive comprehensive obstetric care.

Effects of maternal hypertension on the neonatal haemogram in southern Nigeria: A case-control study.

BACKGROUND: Hypertension in pregnancy is a leading cause of maternal and neonatal morbidity and mortality. This study aimed to compare the hematological parameters in neonates of hypertensive mothers with those of normotensive mothers, and also to compare the incidence of polycythaemia, neutropenia and thrombocytopenia in both groups. METHODS: This was a hospital-based case control study. Three milliliters of cord blood from neonates of women with hypertension in pregnancy and those of normotensive pregnant women were sampled for haemogram parameters using a 3-part autoanalyser. Haematocrit and white blood cell differentials were done manually. Data were analysed using SPSS version 16. RESULTS: A total of 200 neonates were recruited, comprising 100 neonates of mothers with hypertensive disorders of pregnancy and 100 neonates of normotensive mothers. The mean haematocrit was significantly higher in neonates of hypertensive mothers than those of normotensive mothers. The neutrophil and platelet counts of neonates of hypertensive mothers were significantly lower than those of normotensive mothers. The incidences of polycythaemia, neutropenia, and thrombocytopenia were found to be 8%, 15%, and 38% among neonates of hypertensive mothers and 0%, 2%, and 8% among neonates of normotensive mothers, respectively. These incidences were significantly different between the groups. CONCLUSIONS: There was a positive association between hypertension in pregnancy and neonatal polycythaemia, neutropenia, and thrombocytopenia. Haematological parameters of neonates of mothers with hypertension in pregnancy should be properly evaluated and monitored to reduce the chances of developing complications associated with these abnormalities.

Stroke prevalence amongst sickle cell disease patients in Nigeria: a multi-centre study.

BACKGROUND: Stroke is a life-changing, debilitating complication of sickle cell disease (SCD). Previous studies had recorded high stroke prevalence amongst this group of patients. Nigeria has a large population of people affected by this condition and this study aims to assess the stroke prevalence in this large population. METHODOLOGY: Stroke prevalence data from 14 physicians working in 11 tertiary health centres across the country was collated by doctors using the sickle cell registers and patient case notes. This data was then collated and used to obtain the overall stroke prevalence in adult and children. RESULTS: The stroke prevalence in sickle cell disease patients in Nigeria was observed to be 12.4 per 1000 patients. Prevalence in the adult patients was 17.7 per 1000 patients and 7.4 per 1000 patients in children. Twenty three percent of the affected patients had more than stroke episode. CONCLUSION: The stroke prevalence in Nigeria is lower than previously recorded rates and further studies will be required to investigate other factors which may play a role.