大颗粒淋巴细胞白血病与风湿性疾病之间的复杂关系

INTRODUCTION: Large granular lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder characterized by an expansion of clonal T or NK lymphocytes. Neutropenia-related infections represent the main clinical manifestation. Even if the disease follows an indolent course, most patients will ultimately need treatment in their lifetime. Interestingly, LGL leukemia is characterized by a high frequency of autoimmune disorders with rheumatoid arthritis being the most frequent. AREAS COVERED: This review covers the pathophysiology, clinic-biological features and the advances made in the treatment of LGL leukemia.

A special focus will be made on the similarities in the pathophysiology of LGL leukemia and the frequently associated rheumatic disorders. EXPERT OPINION: Recent advances in the phenotypic and molecular characterization of LGL clones have uncovered the key role of JAK-STAT signaling in the pathophysiology linking leukemic cells expansion and autoimmunity.

The description of the molecular landscape of T - and NK-LGL leukemia and the improved understanding of the associated rheumatic disorders open the way to the development of new targeted therapies effective on both conditions.