Coexistence of myeloproliferative neoplasm and plasma-cell dyscrasia.
BACKGROUND
Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), and are characterized by clonal proliferation of hematopoietic cells in the bone marrow. There are numerous case reports and reviews reporting patients with coexisting MPN and plasma-cell disease such as multiple myeloma (MM) and monoclonal gammopathy of undetermined significance (MGUS).


METHODS
We report 15 patients treated at our institution over a 5-year period (January 2008 to December 2012) with a diagnosis of both an MPN and MGUS or MM. We also reviewed and summarized published case reports and studies describing the coexistence of these two disease entities.


RESULTS
Most patients (12/15) had an MPN diagnosis made before or at the same time as the MGUS/MM diagnosis. Eventually, 2 patients developed a lymphoid leukemia, 1 patient developed lymphoma, and 1 patient developed acute myeloid leukemia, raising the question of whether patients with coexistence of myeloid- and lymphoid-derived neoplasms are more prone to leukemic or lymphomatous transformation. We did not find any treatment-related effect that could have contributed to the development of coexisting MGUS or MM and MPN. Of the 7 patients with an abnormal karyotype, 3 patients had trisomy 8.


CONCLUSIONS
At present, management strategies are aimed at treating the MPN and regularly monitoring the MGUS for transformation to an overt plasma-cell malignancy. However, for patients who develop overt MM, management is focused more on treating the myeloma and monitoring the MPN. It has not yet been definitively shown that these 2 entities arise from a common-ancestor hematopoietic stem cell.
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Jyoti
J
Malhotra
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Marina
M
Kremyanskaya
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Emily
E
Schorr
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Ronald
R
Hoffman
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY. Electronic address: john.mascarenhas@mssm.edu.
John
J
Mascarenhas
D006801:Humans; D009196:Myeloproliferative Disorders / Q000175:diagnosis* / Q000473:pathology*; D010265:Paraproteinemias / Q000175:diagnosis* / Q000473:pathology*
Essential thrombocythemia; MGUS; Myelofibrosis; Myeloproliferative neoplasm; Polycythemia vera