[BCR::ABL-Negative Triple Negative Myeloproliferative Neoplasm --Review]

Triple-negative myeloproliferative neoplasms (TN-MPN) are diseases characterized by absence of the three driver mutations in JAK2, CALR, or MPL, but still exhibit histological and phenotypic features sufficient to diagnose myeloproliferative neoplasms (MPN). Approximately 10% to 20% of essential thrombocythemia (ET) and 5% to 10% of primary myelofibrosis (PMF) cases are reported to be triple negative. TN-MPN may carry non-classical driver mutations at JAK2 or MPL, or other gene mutations, including somatic mutations of chromatin structure, epigenetic modifiers (TET2, IDH1/2), splicing factors (SF3B1, SRSF2, U2AF1, ZRSR2), and cytokine signaling regulators (CBL, SH2B3), etc., and there is evidence of clonal hematopoiesis. This article reviews the latest research progress in the pathogenesis, diagnosis, clinical features, prognosis and treatment of TN-MPN.