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The gastro-intestinal microbiota is a key regulator of systemic immunity and inflammatory tone and it contributes to normal haematopoiesis through microbial metabolites, barrier integrity, and host-microbe immune signalling. Disruption of this has been increasingly linked to the development, clinical course, and treatment-related complications of haematological disorders, including clonal haematopoiesis of indeterminate potential (CHIP), leukaemias, and plasma cell neoplasms (PCNs). This review synthesises current evidence on how gut microbiota composition and function intersect with haematopoietic regulation and haematological disease biology. We summarise proposed mechanisms - including microbe-derived metabolites (e.g., short-chain fatty acids), pattern-recognition receptor signalling, intestinal permeability, and cytokine-mediated inflammation - that may influence haematopoietic stem and progenitor cell behaviour and immune cell differentiation. We then discuss disease-specific associations of dysbiosis with CHIP, leukaemias, and PCN, as well as the impact of common haematology interventions (antibiotics, chemotherapy, immunomodulatory therapies, and transplantation) on microbial ecology and downstream clinical outcomes. Finally, we highlight methodological challenges and outline priorities for longitudinal, mechanistic, and multi-omics studies to enable microbiota-informed risk stratification and therapeutic modulation. (1) The gut microbiota influences haematopoiesis via immune signalling, microbial metabolites, and maintenance of mucosal barrier function. (2) Dysbiosis is associated with CHIP, leukaemias, and PCN, and may contribute through chronic inflammation and altered immune homeostasis. (3) Haematological therapies frequently reshape the microbiota; these changes may affect infection risk, treatment tolerance, and outcomes. (4) Current evidence is largely associative; rigorously designed longitudinal and interventional studies are needed to establish causality and guide clinical translation.
Introduction Patients with homozygous β-thalassemia show continuously improved survival, which has contributed to the development of kidney complications, predominantly expressed as increased albuminuria. In particular, patients with transfusion-dependent thalassemia (TDT), frequently develop chronic kidney complications related to chronic anemia, iron overload, and exposure to iron chelation therapy. Although sodium-glucose co-transporter 2 inhibitors (SGLT-2is) have demonstrated renoprotective effects in patients with chronic kidney disease (CKD) from other causes, their efficacy in patients with homozygous β-thalassemia and established CKD remains unknown. Methods In this prospective, open-label study we investigated the impact of dapagliflozin (10 mg/day), in patients with homozygous β-thalassemia and an albumin to creatinine ratio (ACR)>30 mg/g. Study end-points included the effect on ACR and kidney function at 6 months after treatment initiation. Furthermore, we examined the effect on systolic (SBP) and diastolic blood pressure (DBP), and on urine monocyte chemoattractant protein-1 (MCP-1) and matrix metalloproteinase-9 (MMP-9) levels. Results Twelve patients with ACR>30 mg/g received dapagliflozin and 18 patients with normal ACR were followed up for 6 months. Twenty three healthy adult individuals age- and sex-matched to the β-thalassemia group were used as normal controls. Patients with ACR>30 mg/g showed a significant reduction of ACR and SBP at 6 months (p=0.034, p=0.025 respectively) while kidney function remained stable. Patients with homozygous β-thalassemia showed increased baseline urine MCP-1 levels, while MMP-9 showed no differences versus normal controls. Those, who received dapagliflozin exhibited a significant decrease in urine MCP-1 (p=0.022) while MMP-9 remained stable. There were no significant adverse events. Conclusion Dapagliflozin at a standard dose of 10 mg daily is safe and effectively reduces albuminuria and urinary MCP-1 in patients with homozygous β-thalassemia and established CKD, suggesting potential kidney anti-inflammatory effects. Further studies with larger cohorts and longer follow-up are needed to determine its impact on CKD progression and long-term outcomes.
Febrile neutropenia (FN) is a frequent complication following autologous hematopoietic cell transplantation (auto-HCT). Prophylactic use of fluoroquinolones (FQs) has been shown to reduce FN incidence; however, its routine use remains controversial due to increasing concerns regarding antimicrobial resistance and uncertain impact on clinically relevant outcomes. We conducted a retrospective cohort study including adult patients who underwent auto-HCT at A.C.Camargo Cancer Center between 2016 and 2021. Patients were divided into two groups according to institutional prophylaxis policy: those who received levofloxacin prophylaxis during neutropenia between 2016 and 2018 (Px group, n = 201) and those who did not receive FQ prophylaxis between 2018 and 2021 (NPx group, n = 169). The primary outcome was the incidence of FN. Secondary outcomes included microbiologically documented infections, antimicrobial resistance patterns, and 30- and 100-day all-cause mortality. FN occurred more frequently in the NPx group compared with the Px group (92% vs. 81%, p = 0.002). There were no significant differences between groups in the rate of microbiologically documented infections (23% vs. 14%, p = 0.09), 30-day mortality (3% vs. 1%, p = 0.25), or 100-day mortality (3% vs. 2%, p = 0.48). Antimicrobial resistance profiles differed significantly: the Px group had a lower proportion of pan-susceptible isolates (17% vs. 61%) and higher rates of extended-spectrum β-lactamase-producing organisms (27% vs. 6%) and methicillin-resistant bacteria (36% vs. 10%) compared with the NPx group (all p < 0.001). Although FQ prophylaxis was associated with a lower incidence of FN after auto-HCT, it did not translate into reductions in short-term mortality and was associated with significantly higher rates of antimicrobial resistance. Based on these findings, our institution does not recommend routine FQ prophylaxis for patients undergoing auto-HCT.
Chronic myelomonocytic leukemia (CMML) is a biologically heterogeneous myelodysplastic/myeloproliferative neoplasm with limited disease-modifying treatment options beyond hypomethylating agents (HMAs) and allogeneic hematopoietic stem cell transplantation. Venetoclax, a selective BCL-2 inhibitor, is increasingly used off-label in CMML, but its CMML-specific efficacy and safety remain incompletely defined. We conducted a systematic review and meta-analysis in accordance with PRISMA 2020. Eligible studies included adult patients with CMML treated with venetoclax-based regimens and reporting extractable CMML-specific outcomes. PubMed, Embase, Cochrane CENTRAL, conference proceedings, and trial registries were searched through August 2025. Random-effects meta-analyses of proportions were performed for complete remission (CR), marrow complete remission (mCR), and overall response rate (ORR). Seventeen publications representing nine unique studies were included, comprising 145 venetoclax-treated CMML patients. Most regimens combined venetoclax with azacitidine, decitabine, or oral decitabine-cedazuridine, with heterogeneous dosing schedules and frequent CYP3A-guided dose modifications. Responses typically occurred early, within one to two treatment cycles, but durability was generally modest. The pooled CR rate was 19.1% (95% CI: 9.4-34.9; I2 = 55%), the pooled mCR rate was 36.4% (95% CI: 24.7-50.0; I2 = 21%), and the pooled ORR was 71.9% (95% CI: 56.5-83.4; I2 = 56%). Venetoclax-based therapy was associated with substantial myelosuppression, including frequent grade ≥3 neutropenia and thrombocytopenia, with clinically relevant infectious complications. Early mortality was low in studies reporting short-term outcomes. Venetoclax-based regimens demonstrate measurable but limited activity in CMML, with high overall response rates but low CR rates and modest durability. Prospective CMML-specific trials are needed to define optimal dosing, clarify comparative effectiveness, and identify patients most likely to benefit.
Clinical researchers and laboratory specialists are striving to explore artificial intelligence (AI) to facilitate and optimize haematological diagnostics in response to the growing demand for more efficient and accurate diagnoses. This review summarizes current approaches integrating AI into blood and bone marrow cytomorphology, flow cytometry (FC), genetics, and haemostasis. Efforts include automated cell differentiation in peripheral blood and bone marrow aspirates, algorithms for identifying causes of anaemia, tools for rapid diagnosis of acute leukaemia, and other haematological entities. AI in FC may reduce subjectivity and variability, while in genomics, machine learning is increasingly implemented for processing high-throughput sequencing data and may enable automated detection of karyotypes in the future. In haemostasis, AI allows for automation in quality control, the establishment of personalized reference ranges, and potentially automated result interpretation. AI has, however, limitations such as cross-platform compatibility and often lacks sufficient validation. Ethical concerns include risks of bias and regulations are lagging behind the rapid developments. AI shows promise for automating and improving many steps in haematological diagnostics, though final interpretation still needs expert haematologists.
Tagraxofusp, a first-in-class CD123-targeted therapy, is the only approved treatment for blastic plasmacytoid dendritic cell neoplasm (BPDCN). Shared-care models enable coordination and care delivery between specialized cancer treatment centers and local institutions, allowing eligible patients to receive tagraxofusp. This review provides guidance on best practices for effective shared-care implementation for tagraxofusp in patients with BPDCN, including appropriate patient selection, adequate local institutional readiness, proactive collaboration and communication between institutions, integrated co-management between institutions, and requisite staff, patient, and caregiver education. Key Messages: Shared care is particularly suitable for tagraxofusp treatment, as adverse events can be grade 3 or greater, but typically only occur in cycle 1, if they are to occur at all. Based on clinical experience, this article discusses the application of a shared-care model for tagraxofusp treatment in BPDCN.
The Revised International Prognostic Scoring System (IPSS-R) has long been the standard prognostic tool in the management of myelodysplastic syndromes (MDS). Recently, a new clinical-molecular prognostic model, the IPSS-M, was introduced. This model integrates MDS-related gene mutations and offers improved accuracy and precision in predicting patient outcomes and survival. In this study, we compare the prognostic value and predictive capacity of these prognostic scores across different treatment groups. IPSS, IPSS-R, and IPSS-M scores were calculated at diagnosis for all eligible MDS patients in this cohort. Sankey diagrams were generated to visually compare the risk stratification across the three scoring systems. A total of 394 patients were included in this retrospective analysis. During the course of the disease, 59.1% required treatment, with 38.1% (150 patients) receiving erythropoiesis-stimulating agents (ESA) and 19.0% (75 patients) treated with hypomethylating agents (HMA). The IPSS-M was calculated for 281 patients and reclassified 41.9% of cases: 20.3% (n = 57) were upstaged and 21.7% (n = 61) were downstaged. All three scoring systems demonstrated significant stratification of overall survival (OS) in the global cohort (p < 0.001 for IPSS, IPSS-R, and IPSS-M). Notably, IPSS failed to predict OS in patients treated with azacitidine, and none of the scores successfully predicted OS in patients treated with ESA. The IPSS-M effectively reclassified 41.9% of patients, upstaging 20.2% and downstaging 21.7%. However, our analysis found that none of the prognostic scores effectively predicted outcomes for ESA-treated patients. Further studies are needed to assess whether the predictive value of these scoring systems is influenced by the specific treatment modalities used.
This study aimed to characterize multiple myeloma (MM) patients at the first relapse and examine treatment outcomes. This registry-based retrospective study examined relapsed multiple myeloma patients in two Finnish Wellbeing Services Counties (Helsinki and Uusimaa, and Southwest Finland) from 2013 to 2022. Relapse was identified per current guidelines using serum paraprotein, plasma immunoglobulins, and free light chain data. Data were collected from hospital data lakes, Social Insurance Institution and Digital and Population Services Agency. Patients were stratified by age (≤70 vs >70), index year, and time to first relapse (≤2 vs >2 years post-diagnosis). Of 1,221 patients treated for MM between 2012-2022, 286 met the study inclusion criteria. Median overall survival from the relapse was higher in the younger patients (32.4 months vs 20.0 months). In the first half of follow-up, lenalidomide was the most common pharmacotherapy in both age groups. After 2018, CD38-based treatments and pomalidomide increased significantly for younger patients, remaining limited in the elderly. While new therapies have been adopted for younger MM patients, the treatment of older patients at first relapse has had little change. Given their poorer outcomes, there is a need to improve care for older MM patients.
Coronavirus disease 2019 (COVID-19) poses significant risks to immunocompromised individuals. Patients receiving maintenance therapy with anti-CD20 monoclonal antibodies for follicular lymphoma (FL) may be especially vulnerable because of prolonged B-cell depletion. However, data from Japan, particularly from the later stages of the pandemic, remain limited. This study aimed to investigate the incidence, severity, duration, mortality, and treatment discontinuation associated with COVID-19 in this population. We retrospectively analyzed all consecutive 72 patients with FL or related indolent B-cell lymphomas, including transformed FL, who received anti-CD20 maintenance therapy (rituximab or obinutuzumab) at our institute between January 2020 and September 2023. All patients had an indolent lymphoma background. Data on clinical features, laboratory values, disease severity at initial diagnosis according to Ministry of Health, Labour and Welfare guidelines, hospitalization, outcomes, duration of PCR positivity (>21 days defined as prolonged), and maintenance therapy continuation were reviewed. Fisher's exact test was used for analysis. Among the 72 patients (median age: 72 years), 16 (22.2%) developed COVID-19. Of these, 5 had received rituximab and 11 obinutuzumab. The median lymphocyte count at infection was 498/μL; no patient had serum IgG <500 mg/dL. Eight infected patients developed oxygen-requiring disease at initial assessment (50.0% of infected patients; 11.1% overall). Prolonged infection occurred in 9 patients (56.3% of infected patients; 12.5% overall). All four deaths were COVID-19-related and occurred in obinutuzumab recipients (25.0% of infected patients; 5.6% overall). Lymphocyte count <200/μL was associated with mortality (p = 0.027), although this finding should be interpreted cautiously because of the small number of events. Maintenance therapy was discontinued in 5 of 8 patients infected during therapy. COVID-19 infection during anti-CD20 maintenance therapy led to severe illness and prolonged viral shedding in some cases. Severe lymphopenia may be a potential risk marker for mortality, but this finding should be interpreted cautiously because of the exploratory nature of the analysis.
This study evaluated the prognostic role of circular RNAs (circRNAs) in individuals diagnosed with mantle cell lymphoma (MCL), using the framework of the competing endogenous RNA (ceRNA) network model. Differentially expressed circRNAs were identified from the GSE159808 dataset, and differentially expressed messenger RNAs (mRNAs) were extracted from GSE32018. Corresponding microRNAs (miRNAs) were retrieved to construct a ceRNA regulatory network relevant to MCL. Functional enrichment analysis and survival analysis were performed on 35 mRNAs incorporated in the ceRNA network to identify transcripts associated with survival outcomes in MCL. A prognostically relevant ceRNA subnetwork was then constructed based on these results. Thirteen circRNAs (9 upregulated, 4 downregulated) and 457 differentially expressed mRNAs were identified. The initial ceRNA network included 11 circRNAs, 40 miRNAs, and 35 mRNAs. Gene Ontology analysis indicated enrichment in pathways related to dentin-containing tooth development, the plasma membrane signaling receptor complex, and growth factor receptor binding. Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis demonstrated significant involvement in the B-cell receptor signaling pathway, Fc gamma (Fcγ) receptor-mediated phagocytosis, and the Wnt signaling pathway. Survival analysis identified six mRNAs significantly associated with overall survival in individuals with MCL. These results were used to derive a refined prognostic ceRNA network consisting of 6 mRNAs, 7 miRNAs, and 6 circRNAs. CircRNAs may regulate MCL prognosis by modulating miRNA-mRNA interactions within the ceRNA network. These findings suggest a regulatory mechanism by which circRNAs contribute to molecular pathways influencing disease progression and survival in MCL.
The entity of Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) accounts for approximately 20-30% of newly diagnosed adults with B-cell ALL cases in the USA. Compared to other B-cell subtypes, Ph-like ALL is associated with overall poor prognosis and inferior outcomes with high measurable residual disease rates following induction therapy, increased risk of treatment failure and relapse, as well as short event-free and overall survival. Here we aim to highlight Ph-like ALL genetic subtypes and methods of genomic profiling for diagnosis and disease prognostication and to summarize current management approaches for frontline treatment including multiagent chemotherapy, immunotherapy, tyrosine kinase and small molecule inhibitors, and the role of allogeneic stem cell transplantation. Despite the improvement in the treatment outcomes of adult patients with newly diagnosed B-cell ALL, patients with Ph-like ALL continue to do poorly with standard therapy. Thus, tailored therapeutic studies are indeed warranted to refine frontline treatment approaches and to improve outcomes for patients with Ph-like ALL.
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Lymphoma has recovery rates above 60%, but many survivors experience impaired quality-of-life (QoL) requiring survivorship care. This study evaluated the role of an integrative oncology (IO) clinic in managing lymphoma survivors. In this exploratory preference-based controlled trial, adults in remission after lymphoma treatment were allocated to two groups: those attending the IO survivorship clinic (intervention) and those declining (control). The intervention included complementary medicine, spiritual, and social support, delivered weekly for up to 6 months in addition to standard follow-up. The primary outcome was QoL improvement (EQ-5D-5L index). Secondary outcomes included symptom relief (MYCAW), cognitive function, and perceived control. Twenty-nine patients were enrolled: 15 in the intervention and 14 in the control group. Over the first 3 months, a significant time × group interaction in EQ-5D-5L scores favored the intervention (p = 0.005), reflecting superior QoL trajectory. MYCAW concerns also improved significantly in the intervention group across 6 months (p = 0.005 and p = 0.03). At 3 months, FACT Cog-Oth scores were significantly higher in the intervention arm (p = 0.01), indicating better "other" cognitive functions (e.g., memory, clarity, confusion). To note, adherence to IO mainly decreased after 3 months. In this exploratory preference-based study, an IO survivorship clinic for lymphoma survivors was associated with improvements in QoL, especially for adherent patients. Given the preference-based design, these findings should be interpreted cautiously and viewed as hypothesis-generating rather than confirmatory. Further studies are warranted to evaluate long-term benefits and sustainability of this approach.
Improvement in progression-free survival (PFS) is increasingly used by health authorities for cancer drug approvals. We analyzed 106 randomized controlled trials (RCTs) with positive PFS outcomes published in JAMA Oncology, Lancet Oncology, Journal of Clinical Oncology, and Annals of Oncology during 2020 - 2023. RCTs were categorized as 'targeted' (n = 83) or 'conventional' (n = 23) by their interrogated treatment variables. Two measures of PFS effect sizes were analyzed: hazard ratio (HR) and PFS increment. Among the RCTs of targeted therapies, the 25th-percentile value (Q1) of HR increased significantly (slope = 0.05 per year; P = .02) during the study period whilst Q1 of PFS increment decreased significantly (slope = -9.1 percentage points per year; P = .03). Similar trends were not observed among the RCTs of conventional therapies. Our analyses suggest a potential bias in accepting RCTs of targeted therapies despite decreased PFS effect sizes.
Second-generation FLT3 inhibitors have demonstrated clinical efficacy in FLT3-mutated acute myeloid leukemia (AML), although results across randomized trials remain inconsistent. This meta-analysis aimed to evaluate the efficacy of second-generation FLT3 inhibitors in patients with FLT3-mutated AML. We systematically searched databases including PubMed, Web of Science, and the Cochrane Library (from inception to August 2025) to identify randomized controlled trials (RCTs) evaluating second-generation FLT3 inhibitors for treating FLT3-mutated AML. The primary endpoints were overall survival (OS) and progression-free survival (PFS). A random-effects model was used to calculate pooled hazard ratios (HRs) and their 95% confidence intervals (95% CIs). The study protocol was registered in PROSPERO (CRD420251132477). This meta-analysis included eight RCTs involving 2,231 patients. The second-generation FLT3 inhibitors evaluated were gilteritinib and quizartinib, and control treatments included placebo or active non-FLT3-inhibitor regimens. Patients treated with second-generation FLT3 inhibitors exhibited significant improvements in both OS (HR: 0.72, 95% CI: 0.62-0.84; P<0.001) and PFS (HR: 0.73, 95% CI: 0.63-0.86; P<0.001). Subgroup analysis revealed that gilteritinib showed improvements in both OS (HR: 0.71, P=0.001) and PFS (HR: 0.71, P<0.001), and quizartinib significantly improved OS (HR: 0.72, P=0.013) with no significant difference in PFS (HR: 0.75, P=0.142). Among patients with newly diagnosed AML, second-generation FLT3 inhibitors did not significantly improve PFS (HR: 0.73, P=0.127) but significantly improved OS (HR: 0.80, P=0.037). Among patients with relapsed/refractory AML, significant benefits were observed in both OS (HR: 0.67, P<0.001) and PFS (HR: 0.74, P=0.04). Benefits were observed across both placebo-controlled and active-comparator trials. Second-generation FLT3 inhibitors significantly improve survival outcomes in FLT3-mutated AML, particularly for gilteritinib and in relapsed/refractory disease. Further studies are needed to clarify mutation subtype-specific and dose-specific effects.
Acute myeloid leukemia (AML) post myeloproliferative neoplasm (MPN) have very poor prognosis and are often excluded from most clinical trials. We retrospectively collected data from 166 patients, including 156 with available treatment data and 83 with NGS data treated in France and US. 2022 ELN risk categories were favorable, intermediate and adverse in 3 (2%), 17 (13%) and 110 (85%), respectively. ORR was 57%, 20% and 25% in patients treated by intensive chemotherapy (IC), hypomethylating agents (HMA) and BSC (including low intensity treatments as hydroxyurea and low-dose cytarabine), respectively. 31 patients (22%) underwent allogeneic stem cell transplantation (ASCT). Median overall survival (OS) was 7.2 months without significant difference between IC and HMA (9,5 and 8,6 months, respectively). OS was significantly improved in patients allografted (6.7 vs 1.3 months, respectively, p<0.001). Even though 2017 and 2022 ELN risk categories were not prognostic for OS, we observed a prognostic impact on OS of Lindsley's classifier (p=0.014). In multivariate analysis, JAK2 mutation was associated with worse OS (p=0.037), whereas SRSF2 showed a trend toward adverse prognosis (p=0.057). Among functional groups, only spliceosome mutations predicted poor prognosis (p=0.015). In conclusion, we confirmed poor prognosis of AML post MPN. Classical prognostic classification was not validated in our cohort. We observed poor outcome using IC or HMA encouraging us to propose new clinical trials in this specific subgroup. Only ASCT was able to improve prognosis.
As available treatment options for relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL) increase, treatment decisions become increasingly complex. This cross-sectional study evaluated patient and physician decision-making when selecting treatment for R/R CLL in the United States. After an initial pilot, 100 individuals with R/R CLL and 100 physicians who treat R/R CLL completed web-based surveys on factors that influenced treatment selection and levels of involvement during treatment selection. Categorical measures were summarized using numbers/ proportions and continuous measures were summarized using means/standard deviations. Individuals with R/R CLL and physicians prioritized treatment efficacy (individuals: N=47 of 78, 60.3%; physicians: N=79 of 100, 79.0%) and potential side effects (individuals: N=43 of 78, 55.1%; physicians: N=50 of 79, 63.3%) when selecting treatment. Differences in the relative importance of specific factors were observed. Financial and cost factors were important for individuals while medical history was important for physicians. Both cohorts reported a shared decision-making process. Building upon previous findings in the front-line CLL setting, this study confirms that individuals with R/R CLL and physicians commonly consider efficacy and safety of different treatment options when making their decision. Relative importance of specific factors within these and other categories differs across the samples.
The prognosis and management of pregnancy-associated aplastic anemia (PAA) following allogeneic hematopoietic stem cell transplantation (allo-HSCT) remain uncertain. This retrospective study was conducted to describe our clinical experience and to explore the efficacy and safety of allo-HSCT in patients with PAA. A retrospective observational study was evaluated the safety and feasibility of allo-HSCT in 18 patients with PAA who were treated between January 2013 and November 2023. Median neutrophil engraftment occurred at 11.5 days (range: 6-15), and platelet engraftment occurred at 11.5 days (range: 7-28). The cumulative incidence (CuI) of both neutrophil and platelet engraftment was 100%. The CuI rates were 22.96% (95% CI, 13.68-27.32) for grade II acute graft-versus-host disease (aGVHD) and 12.61% (95% CI, 9.32-29.28) for chronic GVHD (cGVHD). No cases of grade III-IV aGVHD, extensive cGVHD, or relapse were observed. The CuI of transplantation-related mortality was 19.87% (95% CI, 13.15-30.73). Among the 15 surviving patients, the median follow-up was 47.0 months (range: 7.6-122.2 months). The overall survival and relapse/rejection-free rates were both 80.14% (95% CI, 69.27-86.86). These findings suggest that allo-HSCT is a viable and effective treatment option for PAA. The donor types included 8 matched sibling donors, 1 matched unrelated donor, 3 mismatched unrelated donors, and 6 haploidentical donors.
Lip lymphoma is an exceptionally rare extranodal lymphoma with limited data to guide clinical management. We conducted the first large-scale population-based analysis to characterize its epidemiology, treatment patterns, and outcomes. We identified all lip lymphoma cases (2000-2022) from the SEER database (17 registries). Patients were categorized as localized primary lip lymphoma (stage I) or lymphoma involving the lip area (stages II-IV). Among 82 patients (median age 62 years, 54.9% female), age-adjusted incidence was 0.0459 per million per year. MALT lymphoma predominated (46.3%). With the median follow-up of 74.5 months, 21 deaths (25.6%) occurred, only 5 (6.1%) lymphoma related. Ten-year overall and cancer-specific survival rates were 72.7% and 92.6%, respectively. In multivariate analysis, age (HR 1.115/year, p < 0.001) and non-low-grade histology (HR 3.251, p = 0.043) independently predicted survival. All lymphoma-related deaths occurred in patients with aggressive or unknown histologies, none in confirmed low-grade B-cell lymphomas. No treatment strategy (surgery, radiotherapy, or systemic treatment) showed a significant superiority in terms of survival. Lip lymphoma demonstrates excellent prognosis, particularly for low-grade B-cell histologies. Age and histological grade represent principal prognostic factors guiding treatment strategies.