Near-total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity.

Abstract

We prospectively studied the efficacy of near total splenectomy (NTS) for managing hereditary spherocytosis (HS) based on haemoglobin (Hb), total bilirubin and splenic remnant regrowth in 30 children receiving NTS for HS between November 1996 and December 2004 (mean followup 3.6 years). Patients were classified into three severity groups. At followup, mean Hb had increased by 2.9-5.0 g/dl (P = 0.110) and bilirubin dropped by 15.4-56.4 mumol/l. Remnant regrowth was moderate, within the weight-specific norm. Amongst spleen-preserving techniques, NTS shows markedly lower rates of recurrent haemolysis, remnant regrowth and secondary operations, thus potentially benefiting all clinical forms of HS.

• Authors: Gerhard A Stoehr, Julian N Sobh, Jan Luecken, Kathrin Heidemann, Uwe Mittler, Reinhard Hilgers, Stefan W Eber
• Journal: British journal of haematology (Br J Haematol) Vol. 132 Issue 6 Pg. 791-3 (Mar 2006) ISSN: 0007-1048 [Print] England
• PMID: 16487182 (Publication Type: Journal Article)

Chemical References

• Hemoglobins
• Bilirubin

Topics

• Adolescent
• Adult
• Bilirubin (blood)
• Child
• Child, Preschool
• Female
• Hemoglobins (analysis)
• Hemolysis (physiology)
• Humans
• Male
• Postoperative Complications
• Postoperative Period
• Prospective Studies
• Risk Factors
• Sepsis (etiology)
• Severity of Illness Index
• Spherocytosis, Hereditary (pathology, physiopathology, surgery)
• Spleen (pathology)
• Splenectomy (methods)
• Treatment Outcome