Rare But Fatal: Hemophagocytic Lymphohistiocytosis (HLH) With Acute Acalculous Cholecystitis

Acalculous cholecystitis is an acute inflammatory disease of the gall bladder with high morbidity and mortality rate. It can be seen in trauma, burns, sepsis, total parenteral nutrition, prolonged fasting, and autoimmune diseases. However, there are very few reports of acalculous cholecystitis with macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) in patients with underlying rheumatic/autoimmune disorders. Here we report a 23-year-old male with a past medical history of granulomatosis with polyangiitis who presented with fever, weight loss, and pancytopenia. A comprehensive infectious evaluation was done including bacterial cultures and viral and fungal serologies. Repeat abdominal imaging obtained later due to developing abdominal pain raised concerns for acute acalculous cholecystitis. Despite aggressive management of sepsis, the patient continued to decline clinically. HLH was suspected when the patient was found to meet the clinical criteria with fever, splenomegaly, cytopenia, hypertriglyceridemia, elevated liver function tests, hypofibrinogenemia, and ferritin of 22K ng/mL, absent NK cell activity, and elevated soluble CD25 receptor levels. Bone marrow biopsy did not reveal hemophagocytosis. Intravenous methylprednisolone was started and the patient showed remarkable clinical improvement with a decrease in all inflammatory markers and did not require any surgical intervention. On the review of the literature, we were able to identify four female patients with underlying adult-onset Still’s disease and Kikuchi disease who presented with HLH along with acalculous cholecystitis likely triggered by flare. Our male patient presented with HLH and acute acalculous cholecystitis. He had a history of granulomatosis polyangiitis (GPA) that remained in remission. Hypersecretion of pro-inflammatory cytokines and cytotoxic cells in HLH promotes ischemia of the gall bladder wall. Early initiation of immunosuppressive therapy under careful observation can prevent surgical intervention and mortality in these patients.