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

Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients / Wong, G. K.; Goldacker, S.; Winterhalter, C.; Grimbacher, B.; Chapel, H.; Lucas, M.; Alecsandru, D.; Mcewen, D.; Quinti, Isabella; Martini, Helene; Schmidt, R. E.; Ernst, D.; Espanol, T.; Vidaller, A.; Carbone, J.; Fernandez Cruz, E.; Lougaris, V.; Plebani, A.; Kutukculer, N.; Gonzalez Granado, L. I.; Contreras, R.; Kiani Alikhan, S.; Ibrahim, M. A. A.; Litzman, J.; Jones, A.; Gaspar, H. B.; Hammarstrom, L.; Baumann, U.; Warnatz, K.; Huissoon, A. P; Clinical Working Party of the European Society for, Immunodeficiencies; Milito, Cinzia. - In: CLINICAL AND EXPERIMENTAL IMMUNOLOGY. - ISSN 0009-9104. - ELETTRONICO. - 172:1(2013), pp. 63-72. [10.1111/cei.12039]

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

QUINTI, Isabella;MARTINI, HELENE;MILITO, CINZIA
2013

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
Autoimmune cytopenia; Common variable immunodeficiency; Splenectomy; Splenomegaly; Adolescent; Adult; Aged; Antibodies, Monoclonal, Murine-Derived; Child; Common Variable Immunodeficiency; Disease Management; Female; Humans; Immunoglobulins; Immunologic Factors; Male; Middle Aged; Retrospective Studies; Rituximab; Splenectomy; Survival Rate; Treatment Outcome; Immunology and Allergy; Immunology
01 Pubblicazione su rivista::01a Articolo in rivista
Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients / Wong, G. K.; Goldacker, S.; Winterhalter, C.; Grimbacher, B.; Chapel, H.; Lucas, M.; Alecsandru, D.; Mcewen, D.; Quinti, Isabella; Martini, Helene; Schmidt, R. E.; Ernst, D.; Espanol, T.; Vidaller, A.; Carbone, J.; Fernandez Cruz, E.; Lougaris, V.; Plebani, A.; Kutukculer, N.; Gonzalez Granado, L. I.; Contreras, R.; Kiani Alikhan, S.; Ibrahim, M. A. A.; Litzman, J.; Jones, A.; Gaspar, H. B.; Hammarstrom, L.; Baumann, U.; Warnatz, K.; Huissoon, A. P; Clinical Working Party of the European Society for, Immunodeficiencies; Milito, Cinzia. - In: CLINICAL AND EXPERIMENTAL IMMUNOLOGY. - ISSN 0009-9104. - ELETTRONICO. - 172:1(2013), pp. 63-72. [10.1111/cei.12039]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/980480
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