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Guidelines for the diagnosis and management of hereditary spherocytosis – 2011 update
Author(s) -
BoltonMaggs Paula H. B.,
Langer Jacob C.,
Iolascon Achille,
Tittensor Paul,
King MayJean
Publication year - 2012
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2011.08921.x
Subject(s) - hereditary spherocytosis , splenectomy , asymptomatic , spherocytosis , concomitant , medicine , gallstones , general surgery , pathology , surgery , spleen
Summary Guidelines on hereditary spherocytosis (HS) published in 2004 (Bolton‐Maggs et al , 2004) are here replaced to reflect changes in current opinion on the surgical management, (particularly the indications for concomitant splenectomy with cholecystectomy in children with mild HS, and concomitant cholecystectomy with splenectomy in those with asymptomatic gallstones). Further potential long term hazards of splenectomy are now recognised. Advances have been made in our understanding of the biochemistry of the red cell membrane which underpins the choice of tests. Biochemical assays of membranes proteins and genetic analysis may be indicated (rarely) to diagnose atypical cases. The diagnostic value of the eosin‐5‐maleimide (EMA) binding test has been validated in a number of studies with understanding of its limitations.