On artherogenic index of plasma in sickle cell anaemia patients.

INTRODUCTION: Sickle cell anaemia (SCA) is an inherited abnormality of haemoglobin associated with reduced life expectancy. Patients' complications include dyslipideamia. This study was aimed at determining the artherogenic index of plasma (AIP) in sickle cell anaemia patients and compares the value to HbAA controls value. A high AIP is strongly predictive of elevated cardiovascular risk. METHODS: A comparative study was conducted among SCA patients attending the haematology clinic, Lagos State University Teaching Hospital (LASUTH) and HbAA Phenotype controls. A total of 304 participants were recruited consisting of equal numbers of SCA and HbAA controls. Single lipid profiles were done; logarithms of triglycerides/high density lipoprotein were calculated to obtain AIP and lipid profile ratios established for all participants. RESULTS: There were lower mean values of Total Cholesterol (TC), High Density Lipoprotein(HDL) and Low Density Lipoprotein (LDL) amongst SCD participants than controls and higher mean values of triglycerides (TG) and Very Low Density Lipoprotein (VLDL) in SCD p < 0.05. The AIP in SCD ranges from -0.62 to 1.32 while that of controls ranges from -0.56 to 0.61.The mean AIP were 0.14 ± 0.29 and -0.009 ± 0.26 in SCD and controls respectively. P value = 0.002. CONCLUSION: AIP value is higher in sickle cell anaemia than controls, the former have lower mean values of TC, HDL and LDL and higher mean values of TG and VLDL.

Haematopoietic stem cell transplantation in Nigerian sickle cell anaemia children patients.

BACKGROUND: Sickle cell anaemia (SCA) remains associated with high risks of morbidity and early death. Children with SCA are at high risk for ischaemic stroke and transient ischaemic attacks, secondary to intracranial arteriopathy involving carotid and cerebral arteries. Allogeneic haematopoietic stem cell transplantation (HSCT) is the only curative treatment for SCA. We report our experience with transplantation in a group of patients with the Black African variant of SCA. PATIENTS AND METHODS: This study included 31 consecutive SCA patients who underwent bone marrow transplantation from human leukocyte antigen (HLA)-identical sibling donors between 2010 and 2014 following a myeloablative-conditioning regimen. RESULTS: The median patient age was 10 years (range 2-17 years). Before transplantation, 14 patients had recurrent, painful, vaso-occlusive crisis; ten patients had recurrent painful crisis in association with acute chest syndrome; three patients experienced ischaemic stroke and recurrent vaso-occlusive crisis; two patients experienced ischaemic stroke; one patient exhibited leukocytosis; and one patient exhibited priapism. Of the 31 patients, 28 survived without sickle cell disease, with Lansky/Karnofsky scores of 100. All surviving patients remained free of any SCA-related events after transplantation. CONCLUSION: The protocols used for the preparation to the transplant in thalassaemia are very effective also in the other severe haemoglobinopathy as in the sickle cell anaemia with 90% disease free survival. Today, if a SCA patient has a HLA identical family member, the cellular gene therapy through the transplantation of the allogeneic haemopoietic cell should be performed. Tomorrow, hopefully, the autologous genetically corrected stem cell will break down the wall of the immunological incompatibility.