Abstract
7103 Background: B-cell chronic lymphocytic leukemia (CLL) results from the accumulation and proliferation of malignant B cells with a predisposition for autoreactivity and autoimmune disease, particularly hematologic disorders such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). We sought to define the prevalence of autoimmune conditions and associated prognostic factors in our CLL cohort. Methods: We retrospectively reviewed the electronic medical records (EMR) of CLL patients at Duke University Medical Center (DUMC) and Durham VA Medical Center (DVAMC) for known diagnoses of autoimmune conditions, or clinical descriptions matching those disorders. Laboratory data confirming the diagnoses (e.g., Coombs test for AIHA) were verified directly in our EMR. IGVH mutation status, CD38 and ZAP-70 expression were previously determined. Results: We found 92 CLL patients with autoimmune disorders (21.4% prevalence), with higher numbers of both hematologic (58, 11.1%) and non-hematologic(42, 8.1%) conditions than previously published data [Barcellini et al. Haematologica (2006) 91:1689], although one series did show 9.7% of CLL patients with hematologic phenomena [Hamblin et al. J Clin Path (1986) 39:713-6]. CLL patients with AIHA and/or ITP had significantly shorter treatment-free survival [2.1 years (1-3.3)] and overall survival [11.9 years (9.6-15.8)] than those without [6.2 years (5.2-7.3) and 18.0 years (14.0-22.6), respectively]. 52% of CLL patients with autoimmunity had mutated IGVH (41/79 known results), 18% were CD38 positive (16/89) and 49% were ZAP-70 positive(39/80). In CLL patients without autoimmune disorders, the prevalence of mutated IGVH, and CD38 and ZAP-70 positivity were similar at 60% (219/364), 25% (99/395), and 47% (176/377), respectively. Conclusions: There is a higher prevalence of both hematologic and non-hematologic autoimmune disorders in our CLL cohort than previously reported. CLL patients with hematologic autoimmune disorders had shorter time to treatment and survival, compared to those without. There was no difference in frequency of mutated IGVH, CD38 or ZAP-70 expression between those patients with autoimmune conditions and those without.
Published Version
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