Long-Term Ovarian Function Assessment After Haematopoietic Stem Cell Transplantation in Female Sickle Cell Anaemia Survivors.

BACKGROUND: Haematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for sickle cell anaemia (SCA). While HSCT offers the possibility of disease remission, it can also lead to long-term complications, including gonadal dysfunction and premature menopause. METHODS: We conducted a retrospective cohort study of female survivors who had hydroxyurea therapy and those who underwent post-HSCT follow-up for SCA at a teaching hospital in Lagos, Nigeria, between January 2019 and December 2022. Participants were eligible if they were at least five years post-HSCT or hydroxyurea treatment and had available serum samples for markers of ovarian function measurement. Demographic and clinical data were collected from the hospital register and patients' medical records. Serum levels of oestradiol, luteinizing hormone (LH), follicle-stimulating hormone (FSH), and anti-Müllerian hormone (AMH) were measured using the Abbott Architect i1000SR chemiluminescent immunoassay analyzer (Abbott Diagnostics, Abbott Park, IL). Descriptive statistics and inferential analyses were used to assess the relationship between markers of ovarian function (FSH and AMH) and clinical parameters. RESULTS: There were statistically significant differences in the median serum levels of all the assessed endocrine hormones between the HSCT and non-HSCT (hydroxyurea) groups of SCA survivors. Up to 82.6% of the SCA survivors experienced ovarian dysfunction after HSCT treatment. Impaired ovarian function in SCA survivors was associated with a longer median follow-up duration than in SCA survivors who had normal ovarian function (12.0 vs. 7.5 years, p = 0.048). There were higher odds of impaired ovarian function in the SCA survivors who had myeloablative regimens than in those who had reduced intensity conditioning regimens (94.1% vs. 50.0%, p = 0.040). CONCLUSION: Our study highlights the significant impact of HSCT on long-term ovarian function in female SCA survivors. However, further prospective studies with larger sample sizes and longer follow-up periods are required to confirm our findings and elucidate the factors influencing ovarian function in SCA survivors of HSCT. In addition, studies are also needed to further elucidate the optimal transplant protocols and fertility preservation strategies to minimize gonadal toxicity and preserve reproductive potential in female SCA patients undergoing HSCT.

Clinical and socio-demographic determinants of pentazocine misuse among patients with sickle cell disease, Benin City, Nigeria: a case-control study.

INTRODUCTION: Opioids are a mainstay in sickle cell disease (SCD) pain care. Opioids are known to cause physical and/or psychological dependence. Increasingly, a significant number of Nigerian SCD patients ("Pentaholics") are observed to abuse pentazocine. This trend is associated with new patterns of medical complications. This study aimed to describe the local spectrum of pentazocine abuse complications and identify possible clinical and socio-demographic determinants. METHODS: We conducted a case control (age matched) study involving 50 booked SCD patients (25 cases and 25 controls) receiving care at the University of Benin Teaching Hospital, Benin City, Nigeria. Relevant clinical and socio-demographic details were collected and analyzed. Associations of categorical variables were tested using chi square or Fishers exact test. RESULTS: The median participants' age and duration of pentazocine abuse/self-use were 32 and 7 years respectively. Pentazocine injections were gotten from local pharmacies and patent medicine stores without any need for physician prescriptions (84% of cases). The buttocks, the thigh and the upper arm/deltoid were the commonest site of injection. Major complications observed were chronic ulcers on the thigh, deep wounds with abscess, healed scar at multiple sites, lower limb swelling and venous thrombosis. Working in healthcare fields/hospitals (Doctor, Nurses, Pharmacists) was significantly associated with pentazocine abuse. CONCLUSION: Health personnel or hospital workers living with SCD are more likely to abuse pentazocine. There is need for prompt triage and optimal control of acute sickle pain, institutional protocols for pain management and strict regulations on supply of prescription drugs such as pentazocine.

Rh and Kell blood group antigen prevalence in a multi-ethnic cohort in Nigeria: implications for local transfusion service.

CONCLUSIONS: Antigens belonging to the Rh and Kell blood group systems are of major clinical significance because of their immunogenicity and the potential of their consequent antibodies to cause in vivo destruction of exogenous red blood cells (RBCs). Despite the wide-spread use of transfusion, there are sparse data on the prevalence of Rh and Kell system antigens and their ethnic variability in Nigeria. The objective of this study was to determine the prevalence of the five major Rh (D, C, c, E, e) and Kell (K) system antigens in Nigeria with the goal of understanding alloimmunization risk in transfusion recipients and improving transfusion safety through the availability of resources, such as antisera for extended RBC typing and antigen panels for alloantibody detection. A multi-ethnic cohort of 302 healthy Nigerian individuals was created to study RBC antigen prevalence. The antigen status of these individuals for Rh and K antigens was determined using commercially prepared antisera and conventional tube agglutination methods. The prevalence of the Rh antigens in the study cohort was found to be: D (92.7%), C (20.5%), c (97.7%), E (19.5%), and e (97.4%). Dce was the most common Rh phenotype (53.3%). The prevalence of K was 0 percent. For all antigens, there was no association between ethnicity and antigen prevalence. This study is the first to document the prevalence of the major Rh and K antigens in the Nigerian population, using a multi-ethnic cohort. Serologic testing demonstrates a zero prevalence of K antigen, which has never been described. C and E pose the higher risks of alloimmunization, hence showing a need for extended RBC typing and matching in at-risk blood recipients. This study demonstrates that phenotyping for major Rh and K antigens within the Nigerian population can potentially improve transfusion safety and prevent alloimmunization.

Hydroxyurea therapy in adult Nigerian sickle cell disease: a monocentric survey on pattern of use, clinical effects and patient's compliance.

BACKGROUND: The clinical prospects of hydroxyurea therapy in the management of sickle cell disease (SCD) require evaluation in the Nigerian setting to develop indigenous guidelines. This survey examines the pattern of hydroxyurea therapy, its clinico-haematologic benefits and safety profile in Nigerian SCD subjects. METHODS: A cross sectional pilot survey was carried out among 60 adult SCD subjects over 3 months. Data on clinical phenotypes, relevant haematological parameters and details of hydroxyurea therapy were obtained using a structured questionnaire through an interview process and case file review. RESULTS: The median age was 30 years. Thirty-four (56.7%) of the subjects are aware of hydroxyurea therapy in SCD. Twenty-four (40%) SCD patients had previously used hydroxyurea. Only 4 subjects were fully compliant. Reasons for non-compliance included poor knowledge and lack of funds. In particular, hydroxyurea reduced leucocyte count and increased mean red cell volume (MCV) in compliant subjects. CONCLUSION: Hydroxyurea use is low among Nigerian SCD subjects despite its proven efficacy/clinical prospects in the developed nations. Large scale multicenter studies and clinical trials are needed to form a basis for developing standard local treatment protocol for its use.

Management of sickle cell disease: a review for physician education in Nigeria (sub-saharan Africa).

Sickle cell disease (SCD) predominates in sub-Saharan Africa, East Mediterranean areas, Middle East, and India. Nigeria, being the most populous black nation in the world, bears its greatest burden in sub-Saharan Africa. The last few decades have witnessed remarkable scientific progress in the understanding of the complex pathophysiology of the disease. Improved clinical insights have heralded development and establishment of disease modifying interventions such as chronic blood transfusions, hydroxyurea therapy, and haemopoietic stem cell transplantation. Coupled with parallel improvements in general supportive, symptomatic, and preventive measures, current evidence reveals remarkable appreciation in quality of life among affected individuals in developed nations. Currently, in Nigeria and other West African states, treatment and control of SCD are largely suboptimal. Improved knowledge regarding SCD phenotypes and its comprehensive care among Nigerian physicians will enhance quality of care for affected persons. This paper therefore provides a review on the aetiopathogenesis, clinical manifestations, and management of SCD in Nigeria, with a focus on its local patterns and peculiarities. Established treatment guidelines as appropriate in the Nigerian setting are proffered, as well as recommendations for improving care of affected persons.

Hypertransfusion therapy in sickle cell disease in Nigeria.

Introduction. Hypertransfusion refers to chronic blood transfusion therapy aimed at ameliorating disease complications in various haemopathies particularly the haemoglobinopathies. In sickle cell disease, hypertransfusion is aimed at maintaining patient's haemoglobin level at 10 to 11 g/dL using haemoglobin AA blood and its resultant dilutional effect on sickle haemoglobin is sustained by intermittent long-term transfusions. Aim and Objective. This paper highlights hypertransfusion and its privileged position as a secondary measure in prevention and treatment of sickle cell disease, especially in the Nigerian context. Materials and Methods. Relevant literatures were searched on PubMed, Google Scholar and standard texts in haematology and transfusion medicine. Keywords used in the search are hypertransfusion, sickle cell disease, chronic transfusion, and Nigeria. Literatures gathered were reviewed, summarized, and presented in this paper. Result. Immense clinical benefit is associated with hypertransfusion therapy including prevention of stroke and amelioration of severe sickle cell disease especially in transplant ineligible patients. Careful patient selections, appropriate blood component, and prevention of transfusion hazards as well as oversight function of an experienced haematologist are pertinent to a successful hypertransfusion therapy. Conclusion. Improved knowledge of the benefits and practice of hypertransfusion will effectively translate into improved health status even among Nigerian sickle cell disease patients.