title: 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.
authors.affiliation: West Midlands Primary Immunodeficiency centre, Birmingham Heartlands Hospital, UK.
authors.forename: G K
authors.initials: GK
authors.lastname: Wong
authors.forename: S
authors.initials: S
authors.lastname: Goldacker
authors.forename: C
authors.initials: C
authors.lastname: Winterhalter
authors.forename: B
authors.initials: B
authors.lastname: Grimbacher
authors.forename: H
authors.initials: H
authors.lastname: Chapel
authors.forename: M
authors.initials: M
authors.lastname: Lucas
authors.forename: D
authors.initials: D
authors.lastname: Alecsandru
authors.forename: D
authors.initials: D
authors.lastname: McEwen
authors.forename: I
authors.initials: I
authors.lastname: Quinti
authors.forename: H
authors.initials: H
authors.lastname: Martini
authors.forename: C
authors.initials: C
authors.lastname: Milito
authors.forename: R E
authors.initials: RE
authors.lastname: Schmidt
authors.forename: D
authors.initials: D
authors.lastname: Ernst
authors.forename: T
authors.initials: T
authors.lastname: Espanol
authors.forename: A
authors.initials: A
authors.lastname: Vidaller
authors.forename: J
authors.initials: J
authors.lastname: Carbone
authors.forename: E
authors.initials: E
authors.lastname: Fernandez-Cruz
authors.forename: V
authors.initials: V
authors.lastname: Lougaris
authors.forename: A
authors.initials: A
authors.lastname: Plebani
authors.forename: N
authors.initials: N
authors.lastname: Kutukculer
authors.forename: L I
authors.initials: LI
authors.lastname: Gonzalez-Granado
authors.forename: R
authors.initials: R
authors.lastname: Contreras
authors.forename: S
authors.initials: S
authors.lastname: Kiani-Alikhan
authors.forename: M A A
authors.initials: MA
authors.lastname: Ibrahim
authors.forename: J
authors.initials: J
authors.lastname: Litzman
authors.forename: A
authors.initials: A
authors.lastname: Jones
authors.forename: H B
authors.initials: HB
authors.lastname: Gaspar
authors.forename: L
authors.initials: L
authors.lastname: Hammarstrom
authors.forename: U
authors.initials: U
authors.lastname: Baumann
authors.forename: K
authors.initials: K
authors.lastname: Warnatz
authors.forename: A P
authors.initials: AP
authors.lastname: Huissoon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000494:pharmacology / Q000627:therapeutic use*; D002648:Child; D017074:Common Variable Immunodeficiency / Q000276:immunology / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D019468:Disease Management; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000494:pharmacology / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab; D013156:Splenectomy; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007136:Immunoglobulins; D007155:Immunologic Factors; D000069283:Rituximab