WONG, G. K., S. GOLDACKER, C. WINTERHALTER, B. GRIMBACHER, H. CHAPEL, M. LUCAS, D. ALECSANDRU, D. MCEWEN, I. QUINTI, H. MARTINI, 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, Jiří LITZMAN, A. JONES, H. B. GASPAR, L. HAMMARSTROM, U. BAUMANN, K. WARNATZ and A. P. HUISSOON. Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients. Clinical and Experimental Immunology. Hoboken: Blackwell Publishing, 2013, vol. 172, No 1, p. 63-72. ISSN 0009-9104. doi:10.1111/cei.12039.
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Basic information
Original name Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients
Authors WONG, G. K. (826 United Kingdom of Great Britain and Northern Ireland), S. GOLDACKER (276 Germany), C. WINTERHALTER (276 Germany), B. GRIMBACHER (276 Germany), H. CHAPEL (826 United Kingdom of Great Britain and Northern Ireland), M. LUCAS (826 United Kingdom of Great Britain and Northern Ireland), D. ALECSANDRU (826 United Kingdom of Great Britain and Northern Ireland), D. MCEWEN (826 United Kingdom of Great Britain and Northern Ireland), I. QUINTI (380 Italy), H. MARTINI (380 Italy), R. E. SCHMIDT (276 Germany), D. ERNST (276 Germany), T. ESPANOL (724 Spain), A. VIDALLER (724 Spain), J. CARBONE (724 Spain), E. FERNANDEZ-CRUZ (724 Spain), V. LOUGARIS (380 Italy), A. PLEBANI (380 Italy), N. KUTUKCULER (826 United Kingdom of Great Britain and Northern Ireland), L. I. GONZALEZ-GRANADO (792 Turkey), R. CONTRERAS (724 Spain), S. KIANI-ALIKHAN (826 United Kingdom of Great Britain and Northern Ireland), M. A. A. IBRAHIM (826 United Kingdom of Great Britain and Northern Ireland), Jiří LITZMAN (203 Czech Republic, guarantor, belonging to the institution), A. JONES (826 United Kingdom of Great Britain and Northern Ireland), H. B. GASPAR (826 United Kingdom of Great Britain and Northern Ireland), L. HAMMARSTROM (752 Sweden), U. BAUMANN (276 Germany), K. WARNATZ (276 Germany) and A. P. HUISSOON (826 United Kingdom of Great Britain and Northern Ireland).
Edition Clinical and Experimental Immunology, Hoboken, Blackwell Publishing, 2013, 0009-9104.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30102 Immunology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 3.278
RIV identification code RIV/00216224:14110/13:00068281
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1111/cei.12039
UT WoS 000316212200008
Keywords in English autoimmune cytopenia; common variable immunodeficiency; splenectomy; splenomegaly
Tags International impact, Reviewed
Changed by Changed by: Ing. Mgr. Věra Pospíšilíková, učo 9005. Changed: 11. 10. 2013 13:01.
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.
Links
7E08062, research and development projectName: Pathophysiology and Natural Course of Primary Antibody Deficiency (PAD) Syndromes
Investor: Ministry of Education, Youth and Sports of the CR, Pathophysiology and Natural Course of Primary Antibody Deficiency (PAD) Syndromes
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