Lymphedema is a rare manifestation of lymphoma: A case series and literature review

Abstract

Lymphedema, which is characterized by impaired lymphatic drainage leading to tissue swelling, represents a relatively uncommon clinical entity, with an estimated prevalence of <1% being observed in the general population. Although most cases arise from postsurgical complications or filariasis, lymphoma-associated lymphedema remains an exceedingly rare manifestation, with only 19 cases documented in the medical literature prior to the present study. The current study presented a case series of 11 patients with histo-logically confirmed lymphoma manifesting with lymphedema, which represents the largest single-center report to date. In the present cohort spanning a time period from 2007-2024, patients who initially presented with refractory lymphedema (9 lower extremity cases, 1 upper extremity case and 1 systemic case) subsequently received a diagnosis of lymphoma via comprehensive evaluation, including imaging (100% detection rate on CT/MRI) and histopathology examinations. The median latency from edema onset to lymphoma diagnosis was 7 months (range, 1-24 months), with 72.7% (8 out of 11) of the patients demonstrating B-cell lineage predominance. The present case series underscored the notion that although lymphedema secondary to lymphoma constitutes <0.5% of all secondary lymphedema cases, it warrants consideration in patients with atypical presentations, such as rapid progression (54.5%), systemic symptoms (81.8%) or abnormal tumor markers (66.7%). The present case report findings emphasized the idea that lymphoma should be included in the differential diagnosis of unexplained lymphedema, particularly when accompanied by warning signs such as lymphadenopathy (100% imaging positivity) or hematologic abnormalities (45.5% anemia prevalence).