[Diffuse B-cell lymphoma associated with hemophagocytic syndrome]

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome of excessive immune activation.
• B-cell lymphoma can present with or trigger HLH, complicating diagnosis and treatment.
• Elderly patients with HLH often have poorer prognoses and unique clinical presentations.

Observation:

• An 84-year-old female presented with fever, hepatosplenomegaly, anemia, and thrombocytopenia.
• Laboratory findings included elevated lactate dehydrogenase (LDH), interleukin-6 (IL-6), and soluble interleukin-2 receptor (sIL-2R).
• Bone marrow biopsy revealed lymphoma cell infiltration and hemophagocytic histiocytes.

Findings:

• Southern blot analysis confirmed gene rearrangements (IgJH and IgJK), indicative of B-cell clonality.
• The patient showed initial improvement with corticosteroids but ultimately died on the 110th hospital day.
• Post-mortem spleen examination confirmed diffuse large B-cell lymphoma with hemophagocytosis.

Implications:

• This case highlights the critical importance of considering secondary HLH in elderly patients with unexplained fever and cytopenias.
• Early recognition and appropriate management of underlying conditions, such as B-cell lymphoma, are crucial for improving outcomes in HLH.
• The aggressive nature of this lymphoma-associated HLH underscores the need for further research into novel therapeutic strategies.