Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients

Abstract

Splenectomy has been used in patients with common variable immunodeficiency disorders (CVID), mainly in the context of refractory autoimmune cytopenia and suspected lymphoma, but there are understandable concerns about the potential of compounding an existing immunodeficiency. With increasing use of rituximab as an alternative treatment for refractory autoimmune cytopenia, the role of splenectomy in CVID needs to be re-examined. This retrospective study provides the largest cohesive data set to date describing the outcome of splenectomy in 45 CVID patients in the past 40 years. Splenectomy proved to be an effective long-term treatment in 75% of CVID patients with autoimmune cytopenia, even in some cases when rituximab had failed. Splenectomy does not worsen mortality in CVID and adequate immunoglobulin replacement therapy appears to play a protective role in overwhelming post-splenectomy infections. Future trials comparing the effectiveness and safety of rituximab and splenectomy are needed to provide clearer guidance on the second-line management of autoimmune cytopenia in CVID.

Splenectomy for immune thrombocytopenia: down but not out

“…Ten patients died, 4 from unusual infections, although two of the four had been on long-term immunosuppression. 99 Splenectomy was not associated with mortality in another cohort of 473 patients with CVID, of which 39 underwent splenectomy with only a single infectious episode in a patient not receiving immunoglobulin. 99 These reports suggest that splenectomy is relatively safe in patients with CVID, despite a small increase in infection risk that may be reduced by immunoglobulin …”

“…Thirty-six patients had autoimmune cytopenias, and 8 of the 11 patients with ITP who underwent splenectomy responded. 99 Nine patients developed overwhelming post-splenectomy infection with encapsulated organisms (infection rate 2.47 per 100 patient years), six of whom had not been receiving intravenous immunoglobulin. Seven of the 9 episodes occurred within 3 years of splenectomy.…”

Resolution of Hypersplenism and Pancytopenia in Common Variable Immunodeficiency Patient After Partial Splenic Artery Embolization: A Case Report and Review of Literature

“…However, there were 16 other episodes of viral reactivations, fungal infections, and other serious bacterial infections, at least four of which were fatal. Overall, the mortality rate in the splenectomized patients was 1.6% per patient year, compared with an estimated rate of 2.3% in all patients with CVID and autoimmune cytopenias [12]. At 6 months follow-up, median (range) platelet counts (134.5 (71.5 -164) × 10 3 /μL) were significantly higher than those before treatment (33.5 (23 -39) × 10 3 /μL; P < 0.05).…”

“…Earlier series reported significant rates of serious infections [8], although later studies are more encouraging. The largest series available analyzed outcomes in 45 patients with CVID from multiple centers who underwent splenectomy, 24 of whom had autoimmune cytopenias [12]. Of these, 19 had failed glucocorticoids, IVIG therapy, or both, and two had failed rituximab.…”

Hypogammaglobulinemia and Common Variable Immunodeficiency

“…105 Splenectomy can be successful even after rituximab has failed. 4,106 Splenectomy is, however, associated with an increased risk of severe infections 11,107 ; therefore, adequate Ig replacement is important during and after this procedure. The treatment for organ-specific autoimmune disorders resembles standard therapy but with an emphasis on minimal immune suppression.…”