A patient with homozygous sickle cell disease presented with sickle crisis complicated by hepatic and pulmonary sequestration and required intensive therapy. During the recovery phrase she developed a rapid rise of haemoglobin concentration, unrelated to blood transfusion, followed by hypertension, congestive cardiac failure and catastrophic intracerebral haemorrhage. This serious haemodynamic ...

Acute splenic sequestration crisis in a 20 year old female with homozygous sickle cell anaemia (Hb SS) is described. The resemblance of this complication to that of splenic vein ligation is discussed. This is the first case report known to the author of acute splenic sequestration crisis in an adult with homozygous sickle cell anaemia treated successfully.

Globally, the majority of persons born with sickle cell disease do not have access to hydroxyurea or more expensive interventions. The objectives were to estimate the survival in homozygous sickle cell disease, unbiased by symptomatic selection and to ascertain the causes of death in a pre-hydroxyurea population. The utility of early life biomarkers and genetically determined phenotypes to pred...

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosi...