Abstract
Acute splenic sequestration (ASS) and chronic hypersplenism are common features of homozygous sickle cell (SS) disease in the first 5 years of life affecting one-third of subjects in the Jamaican Cohort Study. The risk factors are largely unknown and the current study explores a possible role of genetic factors. We have explored these in subjects who received splenectomy in the management of ASS (n=8) or chronic hypersplenism (n=9) along with age, gender, and genotype matched controls using Luminex Technology to assess 42 human cytokines/chemokines, including IL-1α and CXCL10 (IP-10). Levels of IL-1α (p=0.008) and CXCL10 (p=0.009) were significantly elevated in patients treated by splenectomy compared with the control group. Levels of IL-1α were significantly higher in those with a history of ASS compared with matched normal controls (p=0.028) but not in those treated for hypersplenism (p=0.093). Furthermore, several significant differences were found in the median ratios of some cytokine biomarkers between the splenectomized group and the normal controls. These observations are consistent with acute splenic sequestration having a distinct phenotype which may be helpful in predicting those at risk of this complication and suggest that the mechanism of these differences merit further study.
Highlights
Homozygous sickle cell (SS) disease, one of the most common genetic diseases in the world, is associated with marked variability in clinical and hematological features
Serum levels of IL-1a (p = 0.008) and CXCL10 (p = 0.009) were significantly elevated in the splenectomized compared with the control group (Figs 1 A and B)
When values were compared according to the indication for splenectomy, significant differences were confined to IL-1a (p = 0.028) for acute splenic sequestration (ASS) versus controls (Figs 1 C and D)
Summary
Homozygous sickle cell (SS) disease, one of the most common genetic diseases in the world, is associated with marked variability in clinical and hematological features. High levels of fetal hemoglobin protect against sickling early in life, serious complications rapidly develop and the highest mortality occurs at 6–. Foremost among these causes of death are pneumococcal infections and acute splenic sequestration (ASS). The former has been effectively addressed by pneumococcal prophylaxis [5] and the latter by parental education in the early diagnosis of ASS [4]. Hypersplenism emerges as an important complication and differs from ASS with sustained marked splenomegaly and a new hematological equilibrium with lower hemoglobin and higher reticulocyte levels consistent with sustained rapid hemolysis and red cell survival which may be as short as one to three days. The natural history of these complications suggests a median age of one to three years for ASS and three to eight years for chronic hypersplenism
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