BCR-ABL-negative myeloproliferative neoplasms (MPNs) are a group of clonal stem cell disorders characterized by the overproduction of mature myeloid cells. However, MPN patients also have a 2.8- to 3.4-fold increased risk of developing a lymphoproliferative disorder (LPD) compared with the general population.1,2  A range of lymphoid neoplasms have been documented in MPN patients, most commonly chronic lymphocytic leukemia (CLL), which can be diagnosed prior to, simultaneous with, or following the myeloid neoplasm.

The pathogenic mechanism underlying the increased risk of LPD in MPN patients is unknown, but it has been postulated that these disorders could share a common clonal origin. Genetic studies have demonstrated that certain genes, such as SF3B1, TET2, TP53, and BCOR, are mutated in both myelo- and lymphoproliferative diseases, as well as in healthy individuals with age-related...

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