Impact of Binet Staging versus Tumour Bulk on Treatment Outcome in Chronic Lymphocytic Leukaemia.

BACKGROUND: Most of the predictive tools put up to prognosticate treatment outcomes in patients with chronic lymphocytic leukaemia (CLL) are not easily available and affordable in our resource-constrained environment. AIM: The aim of this study was to evaluate the impact of staging and some tumour bulk on treatment outcomes of persons with CLL, Enugu, Nigeria. PATIENTS AND METHODS: This is a 10-year review of the CLL data from the haemato-oncology unit of a Nigerian tertiary hospital to evaluate the impact of staging and tumour bulk indicators. Data were retrieved from the case notes of 102 patients with CLL receiving care at the facility. Data of interest include basic demographic variables, clinical features including spleen size and disease staging and blood counts. Statistical analysis was done using SPSS version 22. RESULTS: The median absolute lymphocyte count (ALC) was 108.05 (confidence interval [CI] = 50.8-201.3, interquartile range [IQR] = 124.4) ×109/L, and duration of survival for the study cohort was 5.5 (CI = 3.5-31.9, IQR = 27) months. Majority (69, 79.3%) were in Stage C. The Binet stage showed a significant association with the ALC (r = 0.338; P = 0.002) but not with spleen size (r = 0.198; P = 0.056). The duration of survival only showed a significant inverse relationship with the ALC (r = 0.35, P = 0.006) but with neither the Binet stage (r = 0.103, P = 0.431) nor spleen size (r = 0.184, P = 0.116). CONCLUSION: In CLL patients, ALC at presentation correlates with the duration of survival. We recommend that the ALC at presentation be used as a prognostic marker in our clime.

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment.

OBJECTIVE: To obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria. DESIGN: A prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV. SETTING: Nigeria. PARTICIPANTS: 308 children (126 girls, 182 boys; age 2-16 years). MAIN OUTCOME MEASURES: Percentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV. RESULTS: In the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was normal in 96 (84%), high in 7 (6%) and conditional in 12 (10%). There were no significant differences in gender or age distribution between the PSV and TAMMV cohorts. Altogether, cerebral artery blood velocity was normal in 246/308 children (80%), high in 14 (4.5%) and conditional in 48 (15.5%). CONCLUSION: Since conditional blood velocity in cerebral arteries can progress to high values and predispose to stroke, the proportion of children with SCD who are affected (15.5%) raises the question of whether regular monitoring and proactive intervention ought to be the standard of care.

Plasma concentration of fucosyltransferase 7 is not associated with the number of clinically overt vaso-occlusive events in sickle cell disease.

Objective: To determine if the number of vaso-occlusive events in SCD relates to plasma concentration of fucosyltransferase 7 (FUT7), which catalyses the synthesis of selectin ligands. Design: A prospective, analytical study. Setting: Haematology and Chemical Pathology Departments of tertiary healthcare centres. Participants: Steady state HbSS individuals aged 13-45 years, 20 had 3 or more vaso-occlusive crises that required hospital admission in the previous year (with or without complications of SCD); 17 other HbSS persons had 0-1 vaso-occlusive crisis that required hospital admission in the previous year and no disease complications. Intervention: Steady-state plasma concentrations of FUT7 measured by ELISA were compared between SCD patients who had one vaso-occlusive crisis requiring hospital treatment in the previous year but no disease complications and those who had >3 crises with or without complications. Main Outcome Measures: Plasma level of FUT7and the number of vaso-occlusive events in each HbSS patient. Results: Mean + standard deviation plasma concentration of FUT7 was 8.6 + 2.7 ng/ml in patients with >3 vasoocclusive crises in the previous year and 7.3 + 1.7 ng/ml in those with 0-1 crisis and no complications; independent sample t-test, p > 0.05, not significantly different. Conclusion: Plasma concentration of fucosyltransferase7 is not associated with the number of vaso-occlusive events in sickle cell disease. Funding: None declared.

Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients.

Sickle leg ulcer (SLU) occurs as a result chronic occlusion of the vasculature with consequent necrosis of the skin and subcutaneous tissue usually in proximity of the malleoli. The description of clinical associations and the simultaneous occurrence of SLU and other complications of SCD compared to the non-SLU patients was the aim of this work. A total of 272 (60.8% males and 39.2% females) patients were captured during this time period out of which 68 (51 males and 17 females) had SLU out of whom 20 patients had bilateral leg ulcers. Prevalence of SLU was 25% and the median age of patients was 25 years, frequency of crisis 2 per annum and 44 (74.6%) had been transfused in the past. Median Hb of the group was 7.6 g/dl and 25% had values lower than 6.5. The occurrence of other complications in SLU patients was as follows; 10 had AVN, 9 priapism, 8 had osteomyelitis, 6 nephropathy, stroke 2, osteoarthritis 4 and cholelithiasis 4. There was a significant relationship between the occurrence of SLU and gender of the patient being more in 67males-Likelihood ratio 4.610 (p = 0.032) and the occurrence of pulmonary hypertension-Likelihood ratio 4.762 (p = 0.029). There was no significant association between the occurrence of SLU and other complications of SCD. Leg ulcer patients have a median age of 25 years with a prevalence of 25% but do not necessarily show other features of severe disease phenotypes. SLU patients were more likely to develop pulmonary hypertension. Further studies on impact of environmental factors on the occurrence of SLU are needed to further evaluate its aetiology.

Low awareness of venous thromboembolism among the general population: a call for increased public enlightenment programs.

BACKGROUND: Venous thromboembolism (VTE) is a notable but often ignored cause of disability and death. Improved public awareness of the symptoms and risks associated with VTE reduces the burden of disease. AIM: We aimed to determine the awareness of VTE among the general population. METHODS: We conducted a population-based study using a pre-tested, pre-validated Ipsos-Reid questionnaire between October 2019 to March 2020. The questionnaire was distributed to consenting adults in the capital cities of Enugu and Ebonyi states of South-Eastern Nigeria to determine their awareness and knowledge of the symptoms and risk factors of VTE. RESULTS: A total of 284 adults participated with a mean age of 32.73 ± 10.33 years and majority (70.8%) had a post-secondary education. While majority were aware of other medical conditions like a heart attack (96.1%), stroke (97.2%), diabetes (98.2%), HIV/AIDS (98.6%), cancer (97.2%) and malaria (98.2), just a few of the subjects were aware of thrombosis (41.5%) and DVT (33.8%). Less than half (42.4%) correctly described DVT as a blood clot in the vein and 13.7% of the respondents knew what PE feels like. A minority of them knew the risk factors of VTE included hospital stay (19.0%), surgery (37.2%), cancer (31.6%), pregnancy (31.6%) and old age (29.6%). Age and gender showed no statistically significant association with awareness of VTE, p value, 0.491 and 0.287, respectively. CONCLUSION: The awareness of VTE in the general population is low. Public awareness programs should be a public health priority to reduce morbidity and mortality associated with VTE.