FRI201 A Rare Case Of Severe Ectopic ACTH Syndrome Presenting With Thrombocytopenia And Hemolytic Anemia

Disclosure: N. Mohammad: None. A. Scott: None. N. Raja-Khan: None. Background: Ectopic ACTH syndrome (EAS) is a rare but serious endocrine emergency requiring urgent diagnostic and therapeutic intervention due to the severity of hypercortisolism induced by ACTH-secreting tumors. Here we present an unusual case of EAS associated with hemolytic anemia and thrombocytopenia. Case Presentation: A 64-year-old female with a history of COPD presented with ongoing fatigue, weakness, and bruising of the extremities. The patient was lethargic, then became hypotensive and encephalopathic. Physical exam revealed diffuse ecchymoses. Patient otherwise had no symptoms or physical stigmata of Cushing’s. Labs were significant for hypokalemia, hemolytic anemia, thrombocytopenia, and lactic acidosis. CT demonstrated consolidation in the right hilum, right posterior lung pleural thickening, hypodense lesion in the right hepatic lobe, and bilateral adrenal hyperplasia. Treatment with antibiotics and plasmapheresis was initiated due to suspicion for thrombotic thrombocytopenic purpura and sepsis. Further workup revealed elevated ACTH 1819pg/mL and AM cortisol 102.4ug/dL. Pituitary MRI was normal. Treatment with etomidate infusion was recommended given the severity of hypercortisolism which was refractory to ketoconazole. However, due to clinical deterioration comfort care was initiated. Autopsy revealed metastatic small cell lung cancer. Conclusion: To our knowledge, this is the first report of EAS presenting with thrombocytopenia and hemolytic anemia. Urgent diagnosis and treatment of hypercortisolism is needed to reduce morbidity and mortality in EAS. While surgical excision of the ACTH-secreting tumor is the mainstay of treatment, cortisol levels should be decreased with medication prior to surgery. In severe cases that are refractory to ketoconazole, etomidate infusion can be considered. Presentation: Friday, June 16, 2023