(TCP003) BILATERAL ASYNCHRONOUS ADRENAL INFARCTION IN JAK2-POSITIVE ESSENTIAL THROMBOCYTHEMIA

Background:

Introduction: BCR-ABL negative myeloproliferative neoplasms (MPNs) are associated with an increased risk of venous and arterial thrombosis at both typical and atypical sites. We report the case of an elderly man diagnosed with bilateral asynchronous adrenal infarction caused by JAK2-positive essential thrombocythemia.

METHODS AND RESULTS:

Case presentation: A 68-year-old man presented to the emergency room (ER) with a recurrent acute abdominal pain. He had a recent history of idiopathic pulmonary embolism with ongoing therapeutic dose of direct oral anticoagulant (Figure 1A). During a recent ER visit for acute abdominal pain, left perirenal infiltration on computed tomography (CT) and mild autoresolutive thrombocytosis of unknown etiology were noted (Figure 1B). During his second ER visit for acute abdominal pain, a repeat abdominal CT was performed and demonstrated left adrenal gland atrophy and right adrenal gland swelling with perirenal infiltration (Figure 1C), suggestive of a bilateral asynchronous adrenalitis. Because of the patient’s history of thrombosis and the acute clinical presentation, a vascular etiology was suspected. After excluding alternative causes of adrenalitis (Table 1), we concluded to a diagnosis of adrenal infarction. During his hospitalisation, he developed a marked thrombocytosis. Bone marrow biopsy and genetic analysis confirmed the diagnosis of JAK2-positive essential thrombocythemia. He is now treated with low molecular weight heparin and hydroxyurea (Figure 1D).

Conclusion:

Conclusion: Essential thrombocythemia may present as adrenal infarction, a rare thrombotic complication and an uncommon cause of adrenalitis. Thrombocytosis, albeit mild, in the context of thromboembolic disease, especially in atypical sites, should be carefully evaluated since diagnosis of essential thrombocythemia changes patient management.