Sickle cell anaemia presents with a dynamic background haemolysis and deepening anaemia. This increases the demand for transfusion if any additional strain on haemopoiesis is encountered due to any other physiological or pathological. Patients with cerebrovascular accident are placed on chronic blood transfusion; those with acute sequestration and acute chest syndrome are likewise managed with blood transfusion. These patients are prone to develop blood transfusion complications including alloimmunization and hyperhaemolytic syndrome (HHS). This term is used to describe haemolysis of both transfused and ‘own’ red cells occurring during or post-transfusion in sickle cell patients. Hyperhaemolysis results in worsening post-transfusion haemoglobin due attendant haemolysis of both transfused and autologous red cells. The mechanism underlying this rare and usually fatal complication of sickle cell has been thought to be secondary to changes in the red cell membrane with associated immunological reactions against exposed cell membrane phospholipids. The predisposition to HHS in sickle cell is also varied and the search for a prediction pattern or value has been evasive. This review to discusses the pathogenesis, risk factors and treatment of hyperhaemolytic syndrome, elaborating what is known of this rare condition.

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Background: Sickle cell Diseases (SCD) is a genetic disorder. It is the most common genetic disorder affecting black race worldwide. It carries a high mortality and morbidity when not promptly and properly managed. Early detection is key to improving the quality of life of people living with sickle cell disease especially in childhood. It has resulted in reduction in mortality rate of SCD to <1% in developed countries through newborn screening but this reduction is yet to be achieved in developing countries. Objective: To share the experience of newborn screening of SCD at COOUTH Awka. To determine the prevalence of Sickle cell disease among neonates in Anambra State. Methodology: A retrospective study of screening for SCD in new-borns carried out at Chukwuemeka Odumegwu Ojukwu University Teaching Hospital (COOUTH) Awka from 1 st September 2013 to 27 th October 2017. This was done using isoelectric focusing machine. Results: A total of 4961 children were screened, 2410 were males, while 2551 were females. 75% (3733) of the neonates had their hemoglobin genotypes as AA, while 0.3% (16) were Hb SS genotype. Prevalence of Hb SS genotype amongst newborns was 0.32%. Conclusion: This study found the prevalence of sickle cell disease in newborns to be 0.32%. Newborn screening using Isoelectric focusing machine is very valuable for early diagnosis and prompt management of SCD. There is therefore need for increased awareness of this in Anambra State.

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.