The effect of the coinheritance of Glucose-6-phosphate dehydrogenase deficiency on the severity of sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are inherited disorders associated with chronic haemolysis. Therefore, coinheritance of both disorders could worsen haemolysis in the former and compound a haemolytic crisis. This study compared clinical and laboratory features of deficient and non-deficient SCD patients and the G6PD activities of SCD patients and apparently healthy controls. MATERIALS AND METHODS: This is a case-control study of 175 SCD patients and 166 non-SCD controls. G6PD assay was carried out on haemolysate from washed red cells. The G6PD activity was measured by spectrophotometry. RESULTS: The mean age of patients and controls was 27.3 ± 9.4 and 35.9 ± 9.7 years, respectively, with 75 (46.2%) and 87 (52.4%) being males, respectively. G6PD activity was similar in cases and controls (6.7 ± 3.3 vs. 6.9 ± 3.0 IU/gHb), respectively (P = 0.6). The prevalence of G6PD deficiency was higher in patients than controls (28.6% vs. 22.3%, P = 0.18), and SCD patients were twice more likely to have enzyme activities below 3.0 IU/gHb. No significant difference was observed in the clinical parameters between deficient and non-deficient patients. Deficient patients were more likely to have lower haematocrit (22.8 ± 3.9% vs. 24.5 ± 5%, P = 0.04) and non-significantly higher bilirubin and reticulocyte counts. Furthermore, in patients, severe deficiency resulted in higher bilirubin than in those with mild deficiency (60.5 vs. 21.7 IU/L, P < 0.001). G6PD activity correlated positively with haematocrit (r = 0.91, P = 0.01) and mean corpuscular haemoglobin concentration (r = 0.17, P = 0.02). CONCLUSIONS: Coinheritance of both disorders could worsen haemolysis in SCD patients, and care should, therefore, be taken in the choice of drugs in deficient SCD patients.

Multiple myeloma with unexplained isolated anaemia in a 24year old man- a case report.

Background: Multiple myeloma (MM) is a disease of the elderly with a median age at presentation of 70 years. It is rare to diagnose MM in individuals less than 40 years and even extremely rare in those less than 30 years of age. MM is usually suspected in those aged 50 years and above having a combination of hypercalcemia, renal insufficiency, anaemia and bone lesions. Although anaemia is a common clinical feature of MM, it is very rare that anaemia would be the only clinical presentation, hence the need to report this index case. Case Presentation: We present a rare case of MM in a 24-year- old male who presented with only symptomatic anaemia. Investigations for the cause of anaemia, including Bone marrow aspiration cytology revealed a diagnosis of MM ISS stage II. Here, we highlighted the need to seek early haematologist consultation in investigating patients' whose cause of anaemia is not immediately obvious from the clinical presentation and routine laboratory investigations. Conclusion: MM can present at a younger age with unexplained anaemia without bone pains or renal insufficiency. High level of suspicion for MM is required in young patients with unexplained anaemia.

CD4+ T Lymphocytes count in sickle cell anaemia patients attending a tertiary hospital.

BACKGROUND: Sickle cell haemoglobin (HbS) is the commonest abnormal haemoglobin and it has a worldwide distribution. Reports have shown that patients with sickle cell anaemia (HbSS) have an increased susceptibility to infection leading to increased morbidity and mortality. Impaired leucocyte function and loss of both humoral and cell-mediated immunity are some of the mechanisms that have been reported to account for the immunocompromised state in patients with sickle cell disease. This study was carried out to determine the CD4+ T lymphocytes count in patients with sickle cell anaemia. MATERIALS AND METHODS: A comparative cross-sectional study of 40 sickle cell anaemia patients in steady state (asymptomatic for at least 4 weeks) attending haematology clinic and 40 age and sex-matched healthy HbA control were recruited into the study. Both HbS patients and the controls were HIV negative. The blood samples obtained were analyzed for CD4+ T cell by Flow cytometry. RESULTS: The study found that there was no significant difference in the number of CD4+ T lymphocyte count between individuals with sickle cell anaemia and HbA (1016 ± 513 cells/µL vs 920 ± 364cells/µL). CONCLUSION: It is recommended that the functionality of CD4+ T lymphocyte should be considered rather than the number in further attempt to elucidate the cellular immune dysfunction in patients with sickle cell anaemia.