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

Area of Science:

• Hematology
• Oncology
• Genetics

Background:

• Triple-negative myeloproliferative neoplasms (TN-MPN) are diagnosed in the absence of JAK2, CALR, or MPL driver mutations.
• These conditions represent 10-20% of essential thrombocythemia and 5-10% of primary myelofibrosis cases.
• TN-MPN exhibit distinct histological and phenotypic characteristics of myeloproliferative neoplasms (MPN).

Purpose of the Study:

• To review the latest research on the pathogenesis of TN-MPN.
• To summarize current knowledge on the diagnosis, clinical features, and prognosis of TN-MPN.
• To discuss emerging treatment strategies for TN-MPN.

Main Methods:

• Literature review of recent studies on TN-MPN.
• Analysis of genetic mutations associated with TN-MPN, including non-classical drivers.
• Synthesis of information on clinical presentation and outcomes.

Main Results:

• TN-MPN can harbor non-classical mutations in JAK2, MPL, or other genes like TET2, IDH1/2, SF3B1, SRSF2, and U2AF1.
• Evidence suggests the presence of clonal hematopoiesis in TN-MPN.
• The review consolidates current understanding of TN-MPN characteristics.

Conclusions:

• TN-MPN represent a complex subgroup of myeloproliferative neoplasms.
• Further research into their unique genetic landscape is crucial for improved diagnostics and therapeutics.
• Understanding TN-MPN pathogenesis is key to developing targeted treatments.