MD Cleveland Clinic Akron General New York, NY, United States
Introduction: Diffuse large B-cell lymphoma (DLBCL) is a common and aggressive non-Hodgkin lymphoma that often presents as rapidly growing lymphoid masses. However, it can occasionally present as hemophagocytic lymphohistiocytosis (HLH). We report a 71-year-old male with undiagnosed DLBCL who initially presented with HLH and multi-organ failure.
Description: A 71-year-old male with a history of diabetes, OSA, hypothyroidism, and hypertension presented with altered mental status. Physical examination revealed lethargy and left neck swelling that had developed over several weeks. Laboratory findings showed leukocytosis, hyperkalemia, anion gap metabolic acidosis, and acute renal failure. Additional results included elevated ferritin (>1650 ng/mL), triglycerides at 664 mg/dL, and a high H-score (>90), indicating a significant likelihood of HLH. Initial treatment in the ED included intravenous fluids and broad-spectrum antibiotics. After stabilization, the patient was admitted to the floor but developed worsening hypoxia, hypotension, and persistent lethargy 12 hours later, leading to transfer to the ICU for septic shock and multi-organ failure of unclear origin. Despite aggressive treatment, including continuous renal replacement therapy (CRRT), vasopressor support, and stress-dose steroids, the patient’s condition worsened, and he was intubated due to deteriorating encephalopathy. Despite extensive efforts, the patient remained in refractory shock. Goals of care discussions led to a transition to do-not-resuscitate (DNR) and comfort measures, and the patient passed away shortly thereafter. Post-mortem evaluation revealed elevated soluble IL-2 receptor levels at 9800 pg/mL, and the cervical lymph node biopsy confirmed DLBCL.
Discussion: This case highlights a rare presentation of DLBCL as HLH with multi-organ failure. HLH, characterized by hyper-inflammatory responses and multi-organ involvement, can be secondary to various conditions, including malignancies. The patient’s high H-score, elevated ferritin, triglycerides, and LDH were consistent with HLH, while the final diagnosis of DLBCL underscores the importance of considering underlying malignancies in such cases. Early identification of malignancy as a potential cause of HLH can significantly influence patient management and outcomes.
