Clinical Report: Coexisting Smoldering Waldenström Macroglobulinemia and MDS
Overview
This report details a rare case of a 74-year-old woman with coexisting smoldering Waldenström macroglobulinemia (SWM) and myelodysplastic syndrome (MDS). The case highlights the importance of reevaluating persistent cytopenias during treatment, as they were attributed to MDS progression rather than SWM.
Background
Waldenström macroglobulinemia (WM) is a low-grade non-Hodgkin lymphoma characterized by monoclonal IgM production, while myelodysplastic syndrome (MDS) involves dysplastic changes in hematopoietic cells and an increased risk of acute myeloid leukemia (AML). The coexistence of these two conditions is extremely rare, making this case significant for clinical understanding and management.
Persistent cytopenias during ibrutinib therapy were due to MDS progression, not SWM.
Clonal evolution was documented from 2.5% blasts to 6% blasts and ultimately to 66% blasts in AML.
The FLT3-ITD mutation emerged post-MDS remission, leading to rapid progression to AML.
The patient initially presented with macrocytic anemia and monoclonal gammopathy.
This case underscores the need for careful monitoring and reevaluation in patients with overlapping hematologic malignancies.
Clinical Implications
Clinicians should maintain a high index of suspicion for MDS in patients with SWM who present with persistent cytopenias. Regular monitoring and reevaluation are crucial to differentiate between disease progression and treatment-related effects.
Conclusion
This case illustrates the complexities of managing patients with coexisting SWM and MDS, emphasizing the need for vigilant clinical assessment and the potential for rapid disease evolution.