Intensivist Chandler Regional Medical Center, Arizona, United States
Introduction: Hemophagocytic lymphohistiocytosis (HLH) requiring ICU have been quoted as having greater than 50% mortality regardless of etiology and treatment. We present a case of rapid presentation, diagnosis and treatment in the ICU.
Methods: A 22 year old previously healthy female presents with fever, nausea, vomiting and epigastric abdominal pain, with a recent diagnosis of Covid. Abdominal CT scan revealed hepatosplenomegaly and sepsis treatment had been initiated with culprit concern of acalculous cholecystitis. She was admitted to the ICU initially requiring low dose pressors with plans for surgical and gastroenterology evaluations with hida scan evaluation. Astute Intensivist evaluation lead to a rapid change in working diagnosis to hemophagocytic lymphohistiocytosis (HLH) given her splenomegaly, fever, pancytopenia, hypofibrinogenemia, and ferritin > 40000ng/ml. Her condition deteriorated to respiratory failure, distributive shock, disseminated intravascular coagulopathy and multisystem organ failure requiring mechanical ventilation and renal replacement therapy. She was started on decadron 10mg bid and IVIG 400mg daily pending bone marrow biopsy which was delayed due to the holiday weekend. However, as her HLH pre-test probability increased and septic shock lowered, bone marrow was demanded and our patient was started on etoposide 125mg daily immediately after marrow was obtained.
Results: Bone marrow revealed trilineage hematopoiesis with erythroid hyperplasia, megakaryocytic hyperplasia, and frequent histiocytes and histiocyte clusters with hemophagocytosis, and later results concluded many histiocytes with positive Ebstein-Barr virus encoded staining. Rutiximab was initiated on hospital day 5, with regimens following the modified HLH-94 protocol during patients' course. She did extremely well, tolerating extubation after a week, and ultimately off oxygen and renal replacement. She was discharged home on hospital day 12, with hematology follow up.
Conclusions: HLH remains an elusive and deadly disease and while engulfed in a myopic world where covid or sepsis alone could easily be the conclusive diagnosis. An ICU provider must remain diligent to their craft and to the broader world around us. We believe aggressive, early clinical diagnosis and treatment remain paramount in the ICU.
