Ryan Duff
Medicine Resident
Madigan Army Medical Center-USASOC
Joint Base Lewis McChord, Washington
Disclosure information not submitted.
Deanna Musfeldt, MD, EM/CCM
Service Chief, Pulmonary, Critical Care and Sleep Medicine
Madigan Army Medical Center, United States
Disclosure information not submitted.
Title: Hidden in Hindsight: A Case of Hemophagocytic Lymphohistiocytosis Following Supplement Ingestion
Introduction: Hemophagocytic Lymphohistiocytosis (HLH) is a rare but serious condition most often seen in the pediatric population. HLH in the adult population is rarer still, and often secondary to malignancy or infection. To date, there have been no reported cases of adult HLH caused by supplement ingestion.
Description: A 30-year-old male without past medical history presented with severe anemia, jaundice, multi-organ failure, and cardiac arrest seven days following daily ingestion of an herbal liver supplement (2Healthy Liver Support). He was admitted to the ICU for hypovolemic/distributive shock requiring blood and pressors following spontaneous splenic hemorrhage. He quickly recovered following treatment with high-dose corticosteroids for hemolytic anemia presumed secondary to the supplement. An extensive workup for infectious, toxic/metabolic, and autoimmune causes for severe anemia, marked leukocytosis, end organ failure, and hyperferritinemia was ultimately negative. Pill analysis was unremarkable. The patient was rapidly weaned off of pressors, extubated, and off antibiotics shortly after admission. His steroid taper was completed after two weeks with markedly improved liver and renal function. However, shortly after discontinuing steroids, he began decompensating again, this time with worsening abdominal pain, tachycardia, and fevers, with negative infectious workup. On hospital day 24, steroids were restarted, and labs were sent for natural killer (NK) cell activity and soluble interleukin-2 (sIL-2) levels to test for HLH. A bone marrow biopsy revealed hemophagocytosis without evidence of malignancy. Tragically, the patient suffered cardiac arrest on hospital day 27, and resuscitation was unsuccessful. Two days later, HLH labs resulted showing absent NK cell activity and elevated sIL-2, consistent with HLH diagnosis.
Discussion: HLH is a rare disorder infrequently seen outside of childhood and is generally associated with hematologic malignancy, genetic predisposition, or infection. We present the case of an otherwise healthy young adult who suffered HLH following supplement use. Prompt and ongoing treatment of the condition is critical, and clinicians must maintain a broad differential to recognize this highly fatal diagnosis.