ABSTRACT

I. INTRODUCTION It has long been understood that chronic lymphocytic leukemia (CLL) is associated with autoimmune disease, almost as a counterpoint to the profound hypogammaglobulinemia that also accompanies it. This chapter takes a fresh look at the original literature and assesses to what extent that understanding reflects the true facts. It recognizes that many of the defining articles were written at a time when CLL was not well distinguished from a number of similar lymphomas. It also attempts to derive an explanation of why autoim­ munity occurs in this context and asks what insights into the pathogenesis of both CLL and autoimmunity the association provides. Finally, it looks at management and considers how secure is current advice.