MDS in Association with Myeloma and MGUS
Yen-Chun Liu, Yue Wu, Susan Mathew, Ruben Niesvizky, Roger Pearse, Atillio Orazi, Scott Ely. Weill-Cornell Medical Center, New York, NY
Background: Recent studies revealed an increased risk of MDS/AML in myeloma (MM) and MGUS. The increased risk in MGUS patients suggested a non-treatment related mechanism. The paucity in characterization of MGUS- or MM-associated MDS adds to the challenges in diagnosing MDS in the presence of plasma cell neoplasms with or without previous treatment. We compiled a series of MGUS- and MM-associated MDS for characterization.
Design: MGUS- (n=6) and MM-associated MDS (n=6) biopsies had karyotypes, MDS-FISH and a full clinical workup.
Results: All but one MGUS-associated MDS were diagnosed concurrently with MGUS at a medium age of 81. In contrast, all MM-associated MDS occurred years after the initial MM diagnosis (median: 13 years) at a median age of 61. All MGUS- and MM-associated MDS showed hypercellularity. Increased blasts were noted in 50% of the MGUS- and 33% of the MM-associated MDS. More MM-(100%) than MGUS (67%)-associated MDS displayed MDS-related cytogenetic changes, among which complex karyotype was more frequent in MM-associated cases (33% vs. 67%). All of the MGUS- and MM-associated MDS had either IgG or IGA paraproteinemia. More CD56 positivity in the plasma cells were found in MM-associated MDS (50% vs 83%). Plasma cell cyclin D1 positivity is not significantly different between these two groups (67% vs 50%). An search identified 5 MM cases with del(20q) with no signs of MDS with follow-up ranging from 0-8 years.
Conclusions: Assessing dysplastic features with an ongoing plasma cell neoplasm can be challenging. The coexistence of MGUS/MM and MDS demonstrates the importance of a complete work-up in plasma cell neoplasm patients developing cytopenias. Along with the clinical presentation and morphologic findings, cytogenetics studies provided additional support for the diagnosis. The negligible time lapse between the MGUS and MDS diagnoses further confirms its non-treatment related nature of association. The median age of diagnosis of MGUS-associated MDS is significantly older than the median age for either MDS or MGUS diagnosis. A small percentage of MM patients were noted to carry MDS-related cytogenetics changes without signs of MDS. Longer follow-up is required to characterize the incidence of MDS in this group of patients.
Wednesday, March 21, 2012 1:00 PM
Poster Session VI # 221, Wednesday Afternoon