Ilginatinib (NS-018) is a selective small-molecule inhibitor of Janus kinase 2 (JAK2), primarily used in the treatment of primary myelofibrosis, a rare bone marrow disorder associated with the JAK2 V617F mutation. It works by inhibiting JAK2, a protein involved in the JAK-STAT signaling pathways that promote the growth of abnormal blood cells. This helps reduce symptoms such as enlarged spleen, fatigue, anemia, and bone pain. Additionally, it is being investigated for its potential in treating other myeloproliferative neoplasms and hematologic malignancies due to its targeted action on dysregulated JAK-STAT pathways. Its selectivity for JAK2 over other JAK family members minimizes off-target effects, making it a promising option for patients with limited treatment alternatives.