Overview: 72-year-old woman on dual immunosuppression (infliximab/Remicade + methotrexate) for seronegative rheumatoid arthritis who developed Epstein-Barr virus (EBV)-associated Hemophagocytic Lymphohistiocytosis (HLH).
On June 5, 2025, admitted for worsening bilateral lower extremity weakness. MRI revealed cord compression at T11-12/T12-L1 and severe cauda equina compression at L3-4. The records document that her presentation included: hyponatremia (Na 123), elevated troponins, diarrhea, and enteritis on imaging (small bowel wall thickening and inflammatory changes on MRI). Discharged to Rehab for PT/OT through 6/18/25.
On June 21, 2025, she was readmitted for FUO (103.1), acute URI, with left shift 92%, tachycardia. Still on dual immunosuppression. Initial assessment was sepsis. She remained hospitalized for 17 days during which time blood cultures were negative, respiratory viral panels negative, Legionella and strep pneumonia antigens negative, CRP elevated, and LFT's rising. She developed ascites, bilateral pleural effusions. An ID consult was done - fever was refractory to four different antibiotic regimens.
Of note, no hematology consult was ordered. No ferritin level was ordered. No EBV or CMV viral load was ordered. No LDH was ordered. No HLH differential diagnosis was considered.
On July 9, 2025, patient was transferred to another facility at the request of decedent' son who is a physician who works in tertiary care. On admission, decedent had markedly elevated inflammatory markers of Ferritin >33,000, ESR 89, CRP 22, LDH 1,507, Hgb 9.5, PLT 125, WBC 14.63. Multiple consultations were obtained including Hem/Onc & treatment for HLH was initiated. Bone marrow biopsy was done. Rituximab given. The next day, client suffered septic shock, Takotsubo cardiomyopathy. She was transitioned to comfort care and passed away on July 14, 2025.
We are evaluating whether earlier hematology consultation and earlier HLH workup/treatment would, within reasonable medical probability, have changed the outcome. Specifically, we need opinions on whether the earlier constellation of fever/systemic illness, cytopenias/lab abnormalities, immunosuppression, enteritis/diarrhea, hyponatremia, and clinical deterioration should have triggered HLH consideration and whether earlier HLH-directed therapy, including rituximab if EBV-associated, would likely have improved survival or avoided the terminal decline.
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