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.
Summary
Splenectomy predisposes for invasive pneumococcal disease. We investigated the immune response of splenectomised hereditary spherocytosis (HS) patients upon sequential pneumococcal vaccination. Thirty‐nine HS‐patients (2‐ to 18‐year‐old) had undergone near‐total or total splenectomy. All received one dose of 7‐valent pneumococcal conjugate vaccine (PCV‐7) and 23‐valent‐pneumococcal‐polysaccharide vaccine (PPV‐23) 2 months apart. Pneumococcal antibodies against serotypes 5/6B/7/14/18C/19F/23F and immunoglobulin serum concentrations were determined before PCV‐7 and 4 weeks after PPV‐23. Significant rises in antibody geometric mean concentrations were observed after PCV‐7 except for serotypes 5 and 7, which increased after PPV‐23. We found no impact of the mode of splenectomy.
Near-total splenectomy provides a favourable immunological basis for natural and vaccine-induced protection against meningococcal serogroup A and C infections. Sequential meningococcal vaccination is immunogenic in patients splenectomized for hereditary spherocytosis.
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