ABSTRACT
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.
Subject(s)
Pneumococcal Infections/prevention & control , Pneumococcal Vaccines/immunology , Spherocytosis, Hereditary/immunology , Splenectomy/adverse effects , Adolescent , Antibodies, Bacterial/analysis , Child , Child, Preschool , Female , Humans , Immunoglobulin A/blood , Immunoglobulin G/blood , Male , Pneumococcal Infections/immunology , Pneumococcal Vaccines/blood , Prospective Studies , Serotyping/methods , Spherocytosis, Hereditary/blood , Spherocytosis, Hereditary/surgery , Splenectomy/methods , Vaccines, Conjugate/immunologyABSTRACT
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.
Subject(s)
Spherocytosis, Hereditary/surgery , Splenectomy/methods , 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 , Spherocytosis, Hereditary/physiopathology , Spleen/pathology , Treatment OutcomeABSTRACT
OBJECTIVE: The authors used a new surgical technique of near-total splenectomy (NTS) and report their experience. SUMMARY BACKGROUND DATA: Total splenectomy is indicated for the management of patients with hereditary spherocytosis but may be complicated by severe infections and thromboembolic events. Studies have shown that partial or subtotal parenchymal resections can lead to excessive regeneration of the residual parenchyma. The resulting onset of hemolysis requires total splenectomy in a significant portion of patients. Our hypothesis was that a more radical approach to open resection permanently decreases recurrent hemolysis while potentially ensuring immune function. METHODS: This longitudinal cohort study included 42 patients with moderate to severe hereditary spherocytosis who underwent NTS according to an open procedure developed by the authors. The end criterion was to conserve a remnant spleen of 10 cm in size. RESULTS: Patient age ranged between 2 and 42 years. Mean resected spleen weight was 580 g; mean remnant volume was 10 cm (range, 8-11 cm). A surgical complication (loss of spleen) occurred in 1 patient. Six-month to 6-year follow-up data was available on 22 patients; 21 of 22 showed preserved phagocytosis and normal blood circulation of the remnant; 1 of 22 experienced secondary remnant necrosis. On average, the remnant spleen grew back to four and a half times its postoperative size. No patients required transfusions, developed gallstones, or symptomatic hemolysis. CONCLUSIONS: This new technique of NTS is safe, effective, and can minimize the late sequelae of secondary splenectomy.
