222: DKA? THINK AGAIN: AN INTERESTING CASE OF HLH

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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of abnormal immune activation that leads to multiorgan dysfunction and death if not diagnosed and treated. Several genetic defects and causative factors have been implicated in the pathogenesis however, presentation with diabetic ketoacidosis (DKA) is uncommon. Description: A 10-year-old male with no past medical history presented to the emergency room for altered mental status, three-day history of nausea and vomiting, and one-day history of abdominal pain and decreased urine output. On exam, the patient was hypothermic, tachycardic, obtunded, and Kussmaul breathing. Initial blood work was consistent with DKA. On hospital day two, while patient’s mental status improved, it had not returned to baseline, and he began having low grade fevers. The patient progressively developed evidence of multiorgan dysfunction with pancytopenia, acute liver failure, hypoxic respiratory failure, coagulopathy, and a hyperinflammatory state including a ferritin level of 1783 ng/mL. The patient’s mental status worsened. Computerized tomography of the brain showed no acute intracranial process; chest radiograph showed evidence of right sided pleural effusion. On day four, patient was transferred to an outside hospital for liver transplant evaluation. The patient subsequently developed evidence of depressed cardiovascular function with an ejection fraction of 38% as well as coronary artery ectasia. Laboratory results were notable for positive GAD Abs consistent with type 1 diabetes mellitus. The patient was found to have diminished natural killer cell function consistent with a diagnosis of HLH. The patient received intravenous immunoglobin, methylprednisolone, and canakinumab as treatment for his HLH. The patient had gradual improvement in his clinical condition and laboratory findings and was discharged home. Discussion: Although the patient initially presented with DKA, he subsequently developed laboratory and physical findings consistent with HLH in addition to his newly diagnosed type 1 diabetes. This case highlights the nonspecific, varied presentation and rapidly progressive nature of HLH. It is important to maintain a broad differential in patients with disease processes that do not respond as expected to treatment.