Prevalence and characteristics of myeloproliferative neoplasms with concomitant monoclonal gammopathy. (November 2020)

• Record Type: Journal Article
• Authors: Javorniczky, Nora Rebeka; Wehrle, Julius; Ihorst, Gabriele; Hupfer, Valerie; Aumann, Konrad; Pfeifer, Dietmar; Niemöller, Christoph; Bleul, Sabine; Pantic, Milena; Werner, Martin; Duyster, Justus; Finke, Jürgen; Engelhardt, Monika; von Bubnoff, Nikolas; Waller, Cornelius F.; Pahl, Heike L.; Becker, Heiko
• Is Part Of: Leukemia research. Volume 98(2020)
• Publication Date: 2020-11
• Subjects: Monoclonal gammopathy -- MGUS -- Myeloproliferative neoplasm -- Primary myelofibrosis -- Essential thrombocythemia -- Polycythemia vera; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2020.106454
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: MGUS prevalence in our cohort of BCR - ABL negative MPN patients was 9 %. No correlation between MGUS and a specific MPN entity or mutation was identified. Our data suggest absence of a strong relationship between MPN and MGUS. In individual patients, MPN and MGUS may feature biological/clonal relationship. Abstract: Of BCR-ABL negative myeloproliferative neoplasm (MPN) patients, 3–14 % display a concomitant monoclonal gammopathy (MGUS). Nonetheless, literature on co-occurring MPN and MGUS is scarce, the molecular underpinnings are unknown and it is unclear whether patients require a specific management. Here, we compared the clinical and genetic features of MPN patients with and without concomitant MGUS. Of 114 MPN patients prospectively studied by serum immunofixation (median age, 67 years; 36.0 % essential thrombocythemia [ET], 24.6 % polycythemia vera [PV], 11.4 % secondary myelofibrosis [sMF], 28.1 % primary myelofibrois [PMF]; 73.7 % JAK2 V617F positive), 10 (9 %) harbored an M-protein. No relevant clinical differences existed between MPN patients with or without M-protein. Seven additional MPN/MGUS patients were retrospectively identified in our MPN registry, yielding a total of 17 patients (7 ET, 3 PV, 3 sMF, 4 PMF). One patient developed multiple myeloma (MM) and one smoldering MM. Seven of 12 patients analyzed carried mutations (e.g. in ASXL1 or TET2 ) in addition to those in JAK2 or CALR, and 4 of 10 patients showed aberrant cytogenetics. M-protein was mainly IgG (12/17), followed by IgM (4/17). In the two patients that underwent allogeneic stem cell transplantation mutant JAK2 and M-protein were no longer detectable post-transplant. In conclusion, MGUS prevalence in our cohort was in the range of previous reports and at most slightly higher than expected in the general population. MGUS presence did not correlate with a specific MPN entity, clinical features or genetic alterations. Our observations suggest that there is no strong clinical or biological relationship between the occurrence of MGUS and MPN.