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Primary hepatic lymphoma HLH represents a rare but critical diagnostic challenge in clinical practice. This condition often masks itself behind non-specific symptoms, frequently leading physicians to suspect more common liver pathologies. Consequently, many cases initially resemble infectious lesions or primary liver cancers. However, early detection remains vital for patient survival.
A 74-year-old woman presented with persistent high fever, fatigue, and loss of appetite for three weeks. Initial physical examinations showed normal vital signs. Imaging studies, including CT, MRI, and FDG-PET/CT, suggested a pyogenic liver abscess. Clinicians eventually performed a laparoscopic liver biopsy to confirm the diagnosis when antibiotic therapy failed to provide relief. Surprisingly, the results revealed germinal center B-cell-like diffuse large B-cell lymphoma (DLBCL).
The clinical course grew complex as the patient developed progressive thrombocytopenia and reduced hemoglobin. These hematological signs, paired with persistent fever, pointed toward secondary hemophagocytic lymphohistiocytosis (HLH). Therefore, the medical team followed the HLH-2004 criteria to confirm this life-threatening complication. This represents a significant finding, as secondary HLH development in PHL is rarely reported in literature.
Treatment began with a modified R-ECHOP chemotherapy regimen to address both the lymphoma and the cytokine storm. Because the patient experienced significant myelosuppression during the first cycle, doctors later switched to the R-miniCHOP protocol. This strategic adjustment provided a safer therapeutic window for the elderly patient while maintaining oncological efficacy. Remarkably, follow-up PET/CT scans showed a complete response after four cycles of treatment.
In conclusion, primary hepatic lymphoma HLH requires high clinical suspicion, especially when a suspected liver abscess fails to respond to standard treatments. Histopathology remains the gold standard for definitive diagnosis. Physicians must recognize that PHL can trigger secondary HLH, much like other hematological malignancies. Furthermore, early recognition and tailored intervention significantly improve the prognosis for these rare and aggressive presentations.
Yes. Due to non-specific imaging features on CT and MRI, PHL often presents as hypodense lesions that closely resemble pyogenic or amebic liver abscesses.
Management usually involves chemo-immunotherapy regimens like R-CHOP or R-ECHOP. In elderly or frail patients, attenuated protocols like R-miniCHOP can effectively balance treatment response and toxicity.
Early biopsy is essential because PHL and HLH are rapidly progressive. A definitive histopathological diagnosis allows for the immediate initiation of life-saving chemotherapy.
Disclaimer: This content is for informational and educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always seek the advice of a qualified healthcare provider regarding any medical condition. Refer to the latest local and national guidelines for clinical practice.
References
1. Zhang Y et al. A Rare Case Report of Primary Hepatic Lymphoma Complicated by Hemophagocytic Lymphohistiocytosis. J Gastrointestin Liver Dis. 2026 Mar 27. doi: 10.15403/jgld-6515. PMID: 41894722.
2. Xu ZY, Pan Y, Tang CL. Primary hepatic lymphoma presenting as pyogenic liver abscess: A case report. World J Clin Cases. 2024;12(21):4742-4747.
3. Peyrade F et al. R-CHOP chemotherapy plus rituximab in patients with diffuse large B-cell lymphoma (DLBCL) aged 80 years or older: a multicentre, single-arm, phase 2 trial (LNH03-7B). Lancet Oncol. 2011;12(5):460-468.
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