Abstract
Forty-five patients with homozygous sickle cell disease who had tonsillectomy for recurrent tonsillitis, when compared with 45 matched controls with haemoglobin genotype AA, showed significant differences in the clinical manifestations and complications of recurrent tonsillitis between the two groups. Although throat swabs in the sickle cell group were mostly negative because they were on prophylactic penicillin, all tonsils harboured Streptococcus pneumoniae when cultured. This study suggests the tonsils to be the more specific source of pneumococcal infection that causes systemic complications which increase morbidity and mortality in sickle cell disease. Although the sickle cell patients may be less clinically symptomatic with tonsillitis, the incidence of serious complications caused by pneumococcal infections, now shown to arise from the tonsils, is significant. Adenotonsillar hypertrophy is linked with an increased risk of a sleep apnoea which causes serious neurological complications such as cerebral infarction and stroke. Tonsillectomy has greatly reduced the incidence of complications from pneumococcal infections in the sickle cell group and should therefore be recommended for sickle cell patients taking prophylactic penicillin and still developing pneumococcal infections.

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