Investigations in the aetiology and pathophysiology of sago haemolytic disease

TL;DRAbstract

Sago haemolytic disease (SHD) is a suspected mycotoxocosis that causes acute and sometime fatal intravascular haemolysis and has only been described in Papua New Guinea (Taufa, 1974; Donovan et al., 1977). Although much is yet to be revealed about this condition, the main risk factor is the consumption of stale sago starch, a food stuff which is the staple carbohydrate for rural Papua New Guineans. It is thought that fungi that colonise the sago are responsible for the haemolytic compound that cleaves red cell membrane proteins. The condition often manifests in family clusters and a genetic predisposition has been postulated. Melanesian Ovalocytosis (MO), also known as South East Asian Ovalocytosis (SAO), is a autosomal recessive genetic disorder expressing a red cell membrane band 3 deletion in individuals who have inherited the gene responsible. The condition is subclinical but common in rural PNG where it is thought to offer some malaria protection (Bruce et al., 2000). It is reason

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