Autoimmune disease in CMML-the chicken or the egg?

Abstract Chronic myelomonocytic leukemia (CMML) is a clonal disorder that is associated with a wide range of systemic inflammatory and autoimmune diseases (SIADs). Approximately 20% of patients with CMML will have an associated SIAD and recognizing this association is critical to the evaluation, prognostication and management of patients with CMML. In this paper, we review the evidence supporting a causative link between these two entities as well as the direction of this relationship. We argue that the data favors CMML as the antecedent and causative disease state with a few notable exceptions. Better understanding of this relationship aids clinicians in the education of their patients and in determining the optimal management approach at the bedside. It is important to recognize opportunities to harmonize the treatments of these disease processes, which may enhance the effectiveness of treatment while reducing the burden of adverse effects from redundant therapies.