Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome often triggered by malignancy, infection, or autoimmune disease. In adults, HLH frequently complicates hematologic cancers such as Hodgkin lymphoma and may mimic sepsis or multiorgan dysfunction. Prompt recognition and treatment are critical as delays are associated with high mortality. Description: A 42-year-old woman with newly diagnosed nodular sclerosing Hodgkin lymphoma presented after syncope with cachexia, malnutrition, and shock requiring vasopressors. She developed progressive jaundice without biliary obstruction, respiratory failure, lactic acidosis, and worsening cytopenias. Norovirus PCR was positive, though gastrointestinal symptoms were absent. Labs showed ferritin 13,059 ng/mL, hemoglobin 8.7 g/dL, platelets 7,000/μL, fibrinogen 160 mg/dL, triglycerides 106 mg/dL, and splenomegaly. Despite absent hypertriglyceridemia, likely from severe malnutrition, HLH was suspected. She received Adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy and steroids; etoposide was withheld due to instability. Renal failure required plasmapheresis and continuous renal replacement therapy, alongside multiple transfusions. Her condition deteriorated, and she died. Postmortem soluble IL-2 receptor (166,750 pg/mL) confirmed HLH. Discussion: This case illustrates the diagnostic challenge of HLH in critically ill, malnourished patients, where laboratory profiles may be atypical. Hypertriglyceridemia, a key criterion, may be blunted in malnutrition. Norovirus, though not a classic trigger, can drive HLH in immunocompromised hosts. Standard treatment for HLH secondary to Hodgkin lymphoma combines corticosteroids and etoposide with malignancy-directed therapy; ABVD alone does not rapidly suppress cytokine storm. Studies show etoposide use improves remission and survival, whereas delays or omission worsen outcomes. Early empiric HLH-directed therapy, ideally before organ failure, and multidisciplinary management, including nutritional optimization, are essential for improving survival.
Soliman et al. (Sun,) studied this question.