A 59 year old man with a history of polycystic kidney disease status post renal transplant presented with a fever. He reported a fever up to 103F, in addition to night sweats and diarrhea. Initial labs were significant for marked transaminitis (ALT/AST 2180/1449 U/L). Work-up of his acute liver injury revealed a significant Ebstein-Barr virus (EBV) viremia (30 million PCR copies/mL). He was also noted to have significant elevations in ferritin (125000 ng/mL) and triglycerides (1000 mg/dL). He was started on rituximab to treat presumed EBV-induced hepatitis. However, the patient began to deteriorate clinically, as he developed worsening pancytopenia, renal function, and liver function. Given his pancytopenia, a bone marrow biopsy was performed, which showed hemophagocytosis. A diagnosis of EBV-associated Hemophagocytic Lymphohistiocytosis (HLH) was made. He was started on etoposide and decadron on hospital day 9. However, he developed progressive multiorgan system failure requiring intubation and initiation of dialysis, and family elected to withdraw care on hospital day 11.
HLH is a rare disorder of abnormal immune activation that is often triggered by underlying infection (especially EBV) or rheumatologic disorder. It presents with fever, cytopenias, high ferritin, and multiorgan dysfunction, usually involving the liver. Diagnosis requires 5 out of 8 criteria: 1. Fever, 2. Splenomegaly, 3. Cytopenia, 4. Fasting triglycerides > 265 mg/dL or fibrinogen < 150 mg/dL, 5. Hemophagocytosis on bone marrow, spleen, lymph node, or liver biopsy, 6. Ferritin > 500 ng/mL, 7. IL-2 receptor activity > 2 standard deviations above normal, and 8. Decreased NK cell activity. The patient in this case met 7 of the 8 criteria (NK cell activity was not checked). Mortality rates are very high in HLH (~50% with treatment), and early treatment with etoposide is key to survival. Therapy should be initiated as soon as possible if high clinical suspicion.
HLH is a life-threatening hematologic disorder of abnormal immune activation that, as in this case, can be triggered by EBV infection. The hospitalist should suspect HLH in setting of unexplained cytopenias, elevated ferritin, and multiorgan dysfunction. Early recognition and treatment is imperative for survival.
To cite this abstract:Schumann C, Duggirala V. Multiorgan Failure Due to Hemophagocytic Lymphohistiocytosis. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 782. https://www.shmabstracts.com/abstract/multiorgan-failure-due-to-hemophagocytic-lymphohistiocytosis/. Accessed December 10, 2018.