Original ArticlesClinicopathologic Features of Myelodysplastic Syndromes Involving Lymph NodesFang, Hong MD*; Wang, Sa A. MD*; Xie, Wei MD, PhD*; Tang, Zhenya MD, PhD*; Hu, Shimin MD, PhD*; Krishnamurthy, Savitri MD†; Medeiros, L. Jeffrey MD*; Wang, Wei MD, PhD*Author Information Departments of *Hematopathology †Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article. Correspondence: Wei Wang, MD, PhD, Department of Hematopathology, Unit 72, MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030 (e-mail: [email protected]). The American Journal of Surgical Pathology: July 2021 - Volume 45 - Issue 7 - p 930-938 doi: 10.1097/PAS.0000000000001689 Buy Metrics Abstract Lymph nodes (LNs) involved by a myelodysplastic syndrome (MDS) are rare and uncommonly biopsied. In this study, we report 6 MDS patients who underwent an LN biopsy that showed MDS, and we summarize the clinicopathologic features of this cohort. All patients presented with lymphadenopathy (generalized in 5), 5 patients had splenomegaly, and 3 patients had hepatomegaly. Histologically, the LN architecture was distorted without complete effacement. MDS cells, mostly of the myeloid lineage, produced interfollicular expansion. These myeloid cells exhibited a spectrum of maturation, and immature and atypical forms were common, including eosinophils. Scattered megakaryocytes and nucleated erythroid cells were often present. Concurrent bone marrow aspirate and biopsy specimens in these patients showed persistent/resistant MDS. Following the diagnosis of LN involvement, patients did not respond well to therapy and all died by the time of the last follow-up, with a median survival of 6.7 months (range, 4.5 to 21.6 mo). In summary, patients with MDS uncommonly develop clinically evident lymphadenopathy prompting biopsy as a result of infiltration by MDS. MDS in LNs can be subtle, showing incomplete and sometimes mild distortion of the architecture, and ancillary studies including immunohistochemical and flow cytometric immunophenotypic analysis are often needed to establish the diagnosis. These data also suggest that the emergence of lymphadenopathy attributable to MDS is associated with poor treatment response and prognosis in MDS patients and that aggressive therapy or alternative treatment regimens need to be explored in this context. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.