Spectrin/band 3 ratio as diagnostic tool in hereditary spherocytosis.
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.
Cutillo
S
S
Department of Paediatrics, University of Naples, Italy.
Pinto
L
L
Nobili
B
B
Miraglia del Giudice
E
E
Iolascon
A
A
D000293:Adolescent; D000328:Adult; D002462:Cell Membrane / Q000737:chemistry; D002469:Cell Separation; D002648:Child; D002675:Child, Preschool; D003720:Densitometry; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D007223:Infant; D008875:Middle Aged; D013049:Spectrin / Q000032:analysis*; D013103:Spherocytosis, Hereditary / Q000097:blood*
D013049:Spectrin