Published online 6 October 2008
Haematologica, Vol 93, Issue 12, 1912-1916 doi:10.3324/haematol.12955
Copyright © 2008 by Ferrata Storti Foundation
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Chronic Lymphocytic Leukemia

Autoimmune conditions and chronic infections in chronic lymphocytic leukemia patients at diagnosis are associated with unmutated IgVH genes

Katrina Vanura1,7, Trang Le1, Harald Esterbauer2, Florentin Späth1, Edit Porpaczy1, Medhat Shehata1,8, Karin Eigenberger1, Alexander Hauswirth1, Cathrin Skrabs1, Elisabeth Krömer3, Ilse Schwarzinger2, Berthold Streubel4,7, Christoph Steininger5, Christa Fonatsch3, Stephan Stilgenbauer6,9, Oswald Wagner2, Alexander Gaiger1,9, Ulrich Jäger1,9

1 Division of Haematology & Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
2 Clinical Institute of Medical & Chemical Laboratory Diagnostics, Medical University of Vienna, Vienna, Austria
3 Department of Medical Genetics, Medical University of Vienna, Vienna, Austria
4 Department of Pathology, Medical University of Vienna, Vienna, Austria
5 Division of Infectious Diseases and Tropical Medicine, Department of Medicine I, Medical University of Vienna, Vienna, Austria
6 Department of Internal Medicine III, University of Ulm, Ulm, Germany
7 Austrian Genome Research Project: Gen-AU-Child
8 K. Landsteiner Institute for Cytokine and Tumour Microenvironment, Vienna, Austria
9 German CLL Study Group

Correspondence: Ulrich Jäger, MD, Division of Haematology & Haemostaseology, Department of Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria. E-mail:ulrich.jaeger{at}meduniwien.ac.at/katrina.vanura{at}meduniwien.ac.at The online version of this article contains a supplementary appendix.

Few data are available concerning the prevalence of autoimmune disease or chronic infections in chronic lymphocytic leukemia patients at diagnosis as well as their clinical outcome. We studied the frequency of such chronic conditions in relation to prognostic markers. A history of autoimmune disease or chronic infection was found in 21% of 186 chronic lymphocytic leukemia patients (12% in autoimmune diseases, 9% in chronic infections). Patients with a history of chronic stimulation were more likely to have unmutated IgVH genes (p<0.002), unfavorable or intermediate risk cytogenetics (11q, 17p deletions, trisomy 12) (p<0.001), and higher CD38 expression (p=0.004). Autoimmune conditions (n=22) were characterized by female predominance (55.0%) with a high frequency of unmutated IgVH (53,8%). Median time to first treatment was 83 months for the chronic stimulation group compared to 128 months for the non-chronic stimulation group (n.s.). Patients suffering from chronic conditions at chronic lymphocytic leukemia diagnosis are likely to have poor prognostic markers, particularly unmutated IgVH genes.

Key words: chronic lymphocytic leukemia, chronic antigenic stimulation, IgVH mutation status, cytogenetics.




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