Evolving Bilateral Adrenal Hemorrhage in Polycythema Vera, Presenting with Acute Severe Hyponatremia and without Hemodynamic Compromise

Presentation Number: SUN 370
Date of Presentation: April 2nd, 2017

Muhammad Z Shah*1, Amir Moheet2 and Lynn A Burmeister1
1University of Minnesota, Minneapolis, MN, 2University of Minnesota Medical Center

Abstract

Background: Bilateral adrenal hemorrhage is a rare occurrence. It usually results in acute adrenal insufficiency, presenting as circulatory collapse. Polycythemia vera (PCV) is a myeloproliferative neoplasm which has risk of both thrombosis and hemorrhage. However bilateral adrenal hemorrhage has been rarely reported in PCV. We present a case of bilateral adrenal hemorrhage in a patient with PCV, with severe hyponatremia and without other typical clinical features of acute adrenal insufficiency.

Clinical Case: 65 years old female presented with right upper quadrant abdominal pain, associated with nausea and vomiting. Past medical history was significant for hypothyroidism, PCV and rheumatoid arthritis. Polycythemia was treated with aspirin and phlebotomies 2-3 times/year. At presentation, vital signs and physical examination were unremarkable except for right upper quadrant abdominal tenderness. Initial laboratory evaluation was unremarkable except for leukocytosis and elevated hematocrit. Abdominal CT was concerning for right adrenal hemorrhage. Additional workup showed serum cortisol of 35 mcg/dl (4-22) and ACTH at the same time was elevated at 77 pg/ml (<47). Serum aldosterone was 4.9 ng/dl with plasma renin of <0.6 ng/ml/hr. Plasma metanephrines were mildly elevated, with normal normetanephrines and DHEAS.

On day 3 of hospitalization, she was noticed to develop mildly low sodium (Na) 130 mmol/L (133-144) which progressively decreased to the lowest reading of 112 mmol/L on day 5. Patient was asymptomatic and hemodynamically stable. Serum potassium was normal. Other laboratory evaluation was consistent with SIADH picture. Repeat morning cortisol was 5 mcg/dl (4-22) and ACTH 121 pg/ml (<47). Repeat plasma metanephrines were normal. Cosyntropin stimulation test showed peak 1 hour serum cortisol of 6.6 mcg/dl. Patient was started on stress dose hydrocortisone for treatment of adrenal insufficiency. She was also placed on free water restriction and briefly received hypertonic saline. Repeat abdominal CT was performed which showed development of a new left adrenal hemorrhage. Na levels continued to improve gradually and were in normal range by day 11. She was discharged home on oral hydrocortisone and fludrocortisone. At 2 week follow up she was clinically well and Na remained normal.

Conclusion: The usual presentation of bilateral adrenal hemorrhage is hypotension or shock (>90 percent of patients). However, our patient was never hemodynamically unstable. On our literature review, there are only three cases reported of bilateral adrenal hemorrhage in PCV, all presenting with circulatory collapse. But our patient’s main clinical problem was severe hyponatremia. Patients presenting initially with unilateral adrenal bleed, should be carefully monitored for possible subsequent progression to bilateral adrenal involvement with risk of acute adrenal insufficiency.

 

Nothing to Disclose: MZS, AM, LAB