SUN-434 - Bilateral Adrenal Hemorrhage in Triple-Negative Essential Thrombocythemia: A Rare Manifestation Unmasking Possible Antiphospholipid Syndrome

Background: Bilateral adrenal hemorrhage (BAH) is a rare but life-threatening condition that can result in acute adrenal insufficiency. It is commonly associated with trauma, sepsis, anticoagulation, or hypercoagulable states, including myeloproliferative neoplasms and antiphospholipid syndrome (APLS). Essential thrombocythemia (ET), particularly in patients with the JAK2V617F mutation, is associated with an increased risk of thrombotic complications. However, vascular events in triple-negative ET are less characterized. We present a rare case of BAH in a patient with triple-negative ET, subsequently found to have positive lupus anticoagulant (LAC).
Clinical

Case: A 68-year-old male with past medical history of triple-negative ET (negative for JAK2, MPL, or CALR mutations), benign prostatic hyperplasia (BPH), and nephrolithiasis presented twice within 2 days to the emergency department with severe nausea/ vomiting, right-sided flank pain, and chills. During the first visit, an abdominal CT scan showed nonspecific mild stranding adjacent to the inferior right adrenal gland. Symptoms were attributed to nephrolithiasis. He was discharged with analgesics. During the second visit, vital signs were stable. Laboratory workup showed mild erythropenia (3.91 M/uL) with increased MCV (106 fL) consistent with hydroxyurea therapy and normal baseline cortisol without a remarkable rise in 30 minutes with cosyntropin stimulation test (cortisol baseline=14.3, n=6-18.4 ug/dL, cortisol 30 mins=16.4 ug/dL, n=4.82-19.50 ug/dL). Aldosterone level was undetectable at < 1 ng/dL with a normal renin activity of 1.05 ng/mL/hr. Abdominal CT-scan revealed interval enlargement of adrenal glands with fat stranding consistent with acute BAH. Despite the absence of overt hypotension, hypoglycemia, or electrolyte disturbances, preemptive stress dose hydrocortisone treatment was started on admission with the concern of evolving adrenal insufficiency over the next few days. The treatment resulted in a significant resolution of symptoms overnight. An extensive hypercoagulability workup was notable for a positive LAC. Patient was discharged with aspirin, hydroxyurea, and a maintenance dose oral hydrocortisone regimen with a plan of outpatient cosyntropin stimulation test. A second set of APLS antibody tests is planned in 12 weeks for a definitive diagnosis of APLS and to determine the need for related anticoagulation.

Conclusion: This case highlights that BAH can also occur in triple-negative ET and underscores the necessity of a broad hypercoagulability workup, even when a primary hematologic disorder is already known. Early recognition and preemptive glucocorticoid therapy are essential to prevent progression to adrenal crisis. Monitoring adrenal reserve with interval cosyntropin stimulation tests should be used to guide the corticosteroid tapering and assess recovery.

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