Spectrin/band 3 ratio as diagnostic tool in hereditary spherocytosis

Eur J Pediatr. 1992 Jan;151(1):35-7. doi: 10.1007/BF02073887.

Abstract

Recently it has been clearly established that partial deficiency of spectrin (SP) evaluated by radioimmunoassay is a common feature of many different forms of hereditary spherocytosis (HS). In this paper the determination of SP density (spectrin/band 3 ratio) in 46 HS patients and in 50 normal controls is presented. The comparison between the membrane SP density of HS subjects and controls showed a statistically significant difference (P less than 0.0005). Moreover no overlap between normal and HS subjects was observed. Membrane spectrin/band 3 ratio has been found related to some clinical features: indeed patients with severe HS showed a smaller SP density than those with milder HS. Our results show that the evaluation of membrane SP density permits a prompt diagnosis of HS and avoids extensive and unnecessary studies for other anaemias.

MeSH terms

  • Adolescent
  • Adult
  • Cell Membrane / chemistry
  • Cell Separation
  • Child
  • Child, Preschool
  • Densitometry
  • Electrophoresis, Polyacrylamide Gel
  • Humans
  • Infant
  • Middle Aged
  • Spectrin / analysis*
  • Spherocytosis, Hereditary / blood*

Substances

  • Spectrin