Figure 1:

Illustration of the factors contributing to development of myeloid neoplasms in autoimmune disease (AD). MNs may be therapy-related (t-MN), occurring after prior cytotoxic or immunosuppressive agent exposure. Leukemogenic potential is highest with thiopurines (azathioprine), alkylators (cyclophosphamide), and topoisomerase inhibitors (mitoxantrone). Important genetic factors influencing t-MN risk are polymorphisms in DNA-repair and drug metabolizing enzymes. Factors likely implicated in MNs arising in AD-treatment naïve patients are shared genetic susceptibilities and/or chronic immune stimulation of the bone marrow environment. * Other ADs such as vasculitides, glomerulonephritides, Behcet’s disease, Sjogren’s syndrome have shown to co-occur and are sometimes the first presenting features of MNs.