Autoimmune conditions and hairy cell leukemia: an exploratory case-control study

Lesley A Anderson,Eric A Engels

doi:10.1186/1756-8722-3-35

Abstract

BackgroundCase reports suggest that hairy cell leukemia (HCL) may be associated with autoimmune conditions, however no systematic investigations in this area have been undertaken.MethodsUsing the United States Surveillance, Epidemiology, and End Results Medicare linked database, we conducted an exploratory study comparing autoimmune conditions in 418 HCL cases (aged ≥65 years) and 160,086 controls.ResultsOverall, the proportion with autoimmune conditions was similar between HCL cases and controls (n = 79 (18.9%) and n = 29,284 (18.3%), respectively). Before diagnosis/selection, there was no overall difference in the prevalence of autoimmune conditions in HCL cases (n = 37, 8.9%) compared with controls (n = 14,085, 8.8%), p = 0.969. However, compared with controls, HCL cases more frequently had sarcoidosis (OR 9.6, 95%CI 2.4-39.5), Sjögren syndrome (OR 6.1, 95%CI 2.0-19.3) and erythema nodosum (OR 37, 95%CI 4.9-284) before diagnosis. Autoimmune conditions were also more common in HCL cases than controls around the time of diagnosis/selection (p < 0.001) but not subsequently.ConclusionsThe findings do not support an overall relationship between autoimmune conditions and HCL, although the association with some autoimmune conditions prior to HCL diagnosis may warrant further investigation. Our findings also suggest that autoimmune conditions in HCL patients may be detected around the time of diagnosis.

Highlights

Hairy cell leukemia (HCL) is a rare, indolent, B-cell neoplasm, accounting for approximately 2% of all nonHodgkin lymphomas (NHLs) in the U.S [1]
Case reports have described the occurrence of autoimmune conditions antecedent to and following diagnosis or treatment of hairy cell leukemia (HCL) [4-8], suggesting that autoimmune conditions may be associated with this malignancy
79 (18.9%) HCL cases and 29,284 (18.3%) controls had at least one of the autoimmune conditions presented in Table 2, p = 0.749

Summary

Introduction

Hairy cell leukemia (HCL) is a rare, indolent, B-cell neoplasm, accounting for approximately 2% of all nonHodgkin lymphomas (NHLs) in the U.S [1]. Organic solvents and some medical conditions including anemia could be related to development of HCL [2]. Factors affecting the immune system, including autoimmune conditions, are associated with an elevated risk of several other NHL subtypes [3]. Case reports have described the occurrence of autoimmune conditions antecedent to and following diagnosis or treatment of HCL [4-8], suggesting that autoimmune conditions may be associated with this malignancy. No prior study has systematically assessed associations between a range of autoimmune conditions. Case reports suggest that hairy cell leukemia (HCL) may be associated with autoimmune conditions, no systematic investigations in this area have been undertaken

Methods

Results

Discussion

Conclusion