Lymphoma-Associated Bone Marrow Hemophagocytosis (LA-BMHPC): A Retrospective, Single-Center Study of 67 Patients

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Objective: To investigate the clinical characteristics, management strategies, and prognostic outcomes of patients with lymphoma-associated bone marrow hemophagocytosis (LA-BMHPC).Methods: This retrospective, single-center cohort study enrolled patients diagnosed with LA-BMHPC between June 1, 2020, and June 30, 2023.We analyzed the clinical characteristics, treatment approaches (hemophagocytic lymphohistiocytosis [HLH]-directed therapy alone [HT], lymphoma-directed therapy alone [LT], simultaneous HLH and lymphoma therapy [HLT], no specific treatment [NST]), and overall survival (OS).Patients meeting the inclusion criteria (n=67) were categorized based on the HLH-2004 criteria into lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH; n=50) and lymphoma-associated hemophagocytosis without fulfilling the HLH criteria (LA-HPC; n=17).Survival was monitored until November 30, 2023.Results: The median overall survival (OS) of the entire LA-BMHPC cohort (n=67) was 3 months (range, 0-40 months), with 3-month, 1-year, and 3-year OS rates of 44.6%, 31.0%, and 22.7%, respectively.Significant differences between LA-HLH and LA-HPC groups were observed in the prevalence of fever, cytopenias (≥2 lineages), hypofibrinogenemia, hyperferritinemia and hypoalbuminemia (all P < 0.05).Receiver operating characteristic analysis identified elevated triglyceride and soluble CD25 levels as strong predictors of progression to LA-HLH, with optimal predictive cut-offs of 1.405 mmol/L and 1352.74U/L, respectively.Multivariate Cox regression analysis revealed that LDH (hazard ratio [HR] 5.991,95% confidence interval [CI], 1.401-25.614;P=0.016), being treatment-naïve at the time of LA-BMHPC diagnosis (HR 2.537, 95% CI, 1.398-4.604;P=0.002), and treatment strategy (overall P=0.001) were independent prognostic factors.Compared to NST, both LT (HR 0.138, 95% CI, 0.046-0.414;P<0.001) and HLT (HR 0.117, 95% CI, 0.069-0.453;P<0.001) were associated with a significantly better survival benefit, whereas HT alone was not (HR 0.450, 95% CI, 0.172-1.180;P=0.104).Patients who received any form of lymphoma-directed therapy (LT or HLT) had significantly better OS than patients who did not (HT or NST; HR = 0.301, 95% CI, 0.160-0.568;P < 0.001).Patients with LA-HPC exhibited a significantly better OS (median, 17 months;1-year rate, 63.7%) than those with LA-HLH (median, 2 months;1-year rate, 20.6%; P=0.015).Conclusions: LA-BMHPC defines a spectrum of diseases ranging from a high-risk precursor state (LA-HPC) to fulminant LA-HLH.Progression to LA-HLH is associated with fever, cytopenias (≥2 lineages), hypofibrinogenemia, hyperferritinemia, hypoalbuminemia, and elevations in triglyceride (≥1.405 mmol/L) or soluble CD25 (≥1352.74U/L) levels.Effective treatment of the underlying lymphoma is the most critical determinant of survival.An integrated strategy (HLT) represents a rational approach, potentially serving as a "bridge" by controlling hyperinflammation to enable definitive anti-lymphoma therapy.

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