Primary Adrenal Insufficiency Due to Recurrent Unilateral Adrenal Infarction in a Patient With Myelodysplastic Syndrome With Isolated del(5q).

BACKGROUND/

OBJECTIVE

Adrenal infarction is a rare cause of primary adrenal insufficiency (PAI), typically associated with hypercoagulable states. While PAI usually results from simultaneous bilateral adrenal involvement, progression from unilateral infarction to bilateral disease leading to PAI is rare. We report the first documented case of adrenal infarction in a patient with myelodysplastic syndrome (MDS) with isolated del(5q), who presented with recurrent unilateral adrenal infarction leading to bilateral involvement and PAI.

CASE DESCRIPTION: A 72-year-old woman with thrombocythemia secondary to MDS with isolated del(5q) on cilostazol therapy presented with left-sided lumbago. Imaging studies, including computed tomography and magnetic resonance imaging, revealed left adrenal infarction. A cosyntropin stimulation test revealed a normal cortisol response. Two months later, the patient developed a second infarction in the right adrenal gland. The patient was diagnosed with PAI confirmed by a low cortisol response to cosyntropin, and started glucocorticoid replacement. Four months later, the patient had a third infarction in the left adrenal gland. Intensifying prophylactic antithrombotic therapy for recurrent adrenal infarction was not considered necessary, as the clinical management would remain unchanged: glucocorticoid replacement for PAI is required regardless of recurrence.

CONCLUSION

We present the first case of recurrent unilateral adrenal infarctions associated with MDS with isolated del(5q), which progressively led to bilateral involvement and PAI. This case suggests the importance of assessing adrenal function and individualized consideration of prophylactic antithrombotic therapy in patients with adrenal infarction.