Momina Zaman, MD,
Resident Physician
n/a, New Jersey, United States
Disclosure information not submitted.
Ahmed Anwar, DO
Resident Physician
Morristown Medical Center, United States
Disclosure information not submitted.
Syed Ahmad, MD
Resident Physician
Morristown Medical Center, United States
Disclosure information not submitted.
Annabeth Johnson, DO
Resident Physician
Morristown Medical Center, United States
Disclosure information not submitted.
Andrew Toscano, MD
Attending Physician
Morristown Medical Center, United States
Disclosure information not submitted.
Title: Acute Abdominal Pain- An Unusual Presentation of Hemophagocytic Lymphohistiocytosis
Case Report Body:
Introduction: Hemophagocytic lymphohistiocystosis (HLH) is an uncommon, life-threatening hyperinflammatory disorder. Its association with pancreatitis is extremely rare, with limited literature available. The delay in HLH diagnosis likely contributes to its high mortality rate. We present the case of a young man with acute abdominal pain and a rapid, unexplained decline.
Description: A 32-year-old man with a BMI of 60 presented to the emergency department with one day of abdominal pain and nausea/vomiting. He had no past medical history or recent alcohol/drug use. He was tachycardic, hypertensive, and hypoxemic. Labs revealed a lipase of 4887 U/L, lactic acid of 5.6 mmol/L, anion gap of 13 mmol/L, triglycerides of 1546 mg/dl, and glucose of 1299 mg/dl. CT scan of the abdomen/pelvis showed hepatic steatosis with hepatosplenomegaly and peripancreatic inflammation. The patient was admitted for further evaluation of presumed hyperosmolar hyperglycemic syndrome and hypertriglyceridemia-induced pancreatitis. Within 12 hours his condition began to rapidly deteriorate, developing severe hyperthermia (Tmax 109°F), fulminant shock, respiratory failure requiring intubation, and acute kidney injury complicated by severe acidosis. Echocardiogram and abdominal exam were unremarkable. Despite fluid resuscitation, antibiotics, vasopressors, stress dose steroids, plasma exchange, and continuous renal replacement therapy, the patient suffered a PEA arrest and expired. Prior to his arrest, a ferritin level obtained was 12722ug/L. Autopsy showed pancreatic necrosis and hepatosplenomegaly without significant ascites; bone marrow examination revealed hemophagocytosis. Based on the above findings, a diagnosis of HLH was made.
Discussion: Diagnosing HLH requires either molecular diagnostic confirmation or fulfillment of 5 of 8 clinical criteria. The diagnosis is challenging due to clinicians’ low index of suspicion and important laboratory tests that are not readily available. In cases like ours, where there is rapid clinical decline and the precipitating illness is not a well-established cause of HLH or is unknown, the challenge is exacerbated. Early screening of septic patients with atypical or severe presentations by checking ferritin levels or HScore may improve time to diagnosis as well as outcomes, and should be further studied.