Abstract
Large granular lymphocytic (LGL) leukemia is a rare form of incurable chronic leukemia frequently complicated by life-threatening cytopenias. The less common NK-cell variant of this disorder poses a diagnostic challenge and its etiologic basis is poorly understood. Here we present the case of an elderly man diagnosed with LGL leukemia after presenting with severe Coombs-negative hemolytic anemia, who had a robust durable response to oral cyclophosphamide. Close to two years after initial diagnosis, he developed a florid Mycobacterium avium-intracellulare (MAI) infection of the lungs. We discuss the clinical and pathologic features of this case, highlighting aspects common to this disorder and areas of clinical uncertainty. We hope to both raise awareness of the risk for pulmonary MAI infection in patients treated with lymphodepleting drugs and to motivate the prospective evaluation of strategies to prevent opportunistic infections in LGL leukemia.