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

G K Wong; S Goldacker; C Winterhalter; B Grimbacher; H Chapel; M Lucas; D Alecsandru; D McEwen; I Quinti; H Martini; C Milito; R E Schmidt; D Ernst; T Espanol; A Vidaller; J Carbone; E Fernandez-Cruz; V Lougaris; A Plebani; N Kutukculer; L I Gonzalez-Granado; R Contreras; S Kiani-Alikhan; M A A Ibrahim; J Litzman; A Jones; H B Gaspar; L Hammarstrom; U Baumann; K Warnatz; A P Huissoon; Clinical Working Party of the European Society for Immunodeficiencies (ESID); Gabriel Wong

doi:10.1111/cei.12039

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

G K Wong, S Goldacker, C Winterhalter, B Grimbacher, H Chapel, M Lucas, D Alecsandru, D McEwen, I Quinti, H Martini, C Milito, R E Schmidt, D Ernst, T Espanol, A Vidaller, J Carbone, E Fernandez-Cruz, V Lougaris, A Plebani, N KutukculerL I Gonzalez-Granado, R Contreras, S Kiani-Alikhan, M A A Ibrahim, J Litzman, A Jones, H B Gaspar, L Hammarstrom, U Baumann, K Warnatz, A P Huissoon, Clinical Working Party of the European Society for Immunodeficiencies (ESID), Gabriel Wong

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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.

Original language	English
Pages (from-to)	63-72
Number of pages	10
Journal	Clinical & Experimental Immunology
Volume	172
Issue number	1
DOIs	https://doi.org/10.1111/cei.12039
Publication status	Published - Apr 2013

Bibliographical note

Keywords

Adolescent
Adult
Aged
Antibodies, Monoclonal, Murine-Derived
Child
Common Variable Immunodeficiency
Disease Management
Female
Humans
Immunoglobulins
Immunologic Factors
Male
Middle Aged
Retrospective Studies
Splenectomy
Survival Rate
Treatment Outcome

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10.1111/cei.12039

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Wong, G. K., Goldacker, S., Winterhalter, C., Grimbacher, B., Chapel, H., Lucas, M., Alecsandru, D., McEwen, D., Quinti, I., Martini, H., Milito, C., Schmidt, R. E., Ernst, D., Espanol, T., Vidaller, A., Carbone, J., Fernandez-Cruz, E., Lougaris, V., Plebani, A., ... Wong, G. (2013). Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients. Clinical & Experimental Immunology, 172(1), 63-72. https://doi.org/10.1111/cei.12039

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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.",

keywords = "Adolescent, Adult, Aged, Antibodies, Monoclonal, Murine-Derived, Child, Common Variable Immunodeficiency, Disease Management, Female, Humans, Immunoglobulins, Immunologic Factors, Male, Middle Aged, Retrospective Studies, Splenectomy, Survival Rate, Treatment Outcome",

author = "Wong, {G K} and S Goldacker and C Winterhalter and B Grimbacher and H Chapel and M Lucas and D Alecsandru and D McEwen and I Quinti and H Martini and C Milito and Schmidt, {R E} and D Ernst and T Espanol and A Vidaller and J Carbone and E Fernandez-Cruz and V Lougaris and A Plebani and N Kutukculer and Gonzalez-Granado, {L I} and R Contreras and S Kiani-Alikhan and Ibrahim, {M A A} and J Litzman and A Jones and Gaspar, {H B} and L Hammarstrom and U Baumann and K Warnatz and Huissoon, {A P} and {Clinical Working Party of the European Society for Immunodeficiencies (ESID)} and Gabriel Wong",

note = "{\textcopyright} 2012 British Society for Immunology.",

year = "2013",

month = apr,

doi = "10.1111/cei.12039",

language = "English",

volume = "172",

pages = "63--72",

journal = "Clinical & Experimental Immunology",

issn = "0009-9104",

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Wong, GK, Goldacker, S, Winterhalter, C, Grimbacher, B, Chapel, H, Lucas, M, Alecsandru, D, McEwen, D, Quinti, I, Martini, H, Milito, C, Schmidt, RE, Ernst, D, Espanol, T, Vidaller, A, Carbone, J, Fernandez-Cruz, E, Lougaris, V, Plebani, A, Kutukculer, N, Gonzalez-Granado, LI, Contreras, R, Kiani-Alikhan, S, Ibrahim, MAA, Litzman, J, Jones, A, Gaspar, HB, Hammarstrom, L, Baumann, U, Warnatz, K, Huissoon, AP, Clinical Working Party of the European Society for Immunodeficiencies (ESID) & Wong, G 2013, 'Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients', Clinical & Experimental Immunology, vol. 172, no. 1, pp. 63-72. https://doi.org/10.1111/cei.12039

TY - JOUR

T1 - Outcomes of splenectomy in patients with common variable immunodeficiency (CVID)

T2 - a survey of 45 patients

AU - Wong, G K

AU - Goldacker, S

AU - Winterhalter, C

AU - Grimbacher, B

AU - Chapel, H

AU - Lucas, M

AU - Alecsandru, D

AU - McEwen, D

AU - Quinti, I

AU - Martini, H

AU - Milito, C

AU - Schmidt, R E

AU - Ernst, D

AU - Espanol, T

AU - Vidaller, A

AU - Carbone, J

AU - Fernandez-Cruz, E

AU - Lougaris, V

AU - Plebani, A

AU - Kutukculer, N

AU - Gonzalez-Granado, L I

AU - Contreras, R

AU - Kiani-Alikhan, S

AU - Ibrahim, M A A

AU - Litzman, J

AU - Jones, A

AU - Gaspar, H B

AU - Hammarstrom, L

AU - Baumann, U

AU - Warnatz, K

AU - Huissoon, A P

AU - Clinical Working Party of the European Society for Immunodeficiencies (ESID)

AU - Wong, Gabriel

PY - 2013/4

Y1 - 2013/4

N2 - 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.

AB - 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.

KW - Adolescent

KW - Adult

KW - Aged

KW - Antibodies, Monoclonal, Murine-Derived

KW - Child

KW - Common Variable Immunodeficiency

KW - Disease Management

KW - Female

KW - Humans

KW - Immunoglobulins

KW - Immunologic Factors

KW - Male

KW - Middle Aged

KW - Retrospective Studies

KW - Splenectomy

KW - Survival Rate

KW - Treatment Outcome

U2 - 10.1111/cei.12039

DO - 10.1111/cei.12039

M3 - Article

C2 - 23480186

SN - 0009-9104

VL - 172

SP - 63

EP - 72

JO - Clinical & Experimental Immunology

JF - Clinical & Experimental Immunology

IS - 1

ER -

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

Abstract

Bibliographical note

Keywords

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