The prognosis of patients with myelodysplastic neoplasms（MDS）is closely linked to their cytogenetic and molecular genetic characteristics. Among the most prevalent genetic mutations in MDS，the TP53 gene abnormalities stand out as an independent adverse prognostic factor for MDS and a significant risk factor for the progression to acute myeloid leukemia（AML）. The recently updated 5th edition of the WHO classification of haematolymphoid tumours（WHO 2022）and international consensus classification of myeloid neoplasms and acute leukemia（ICC）recognizes MDS with TP53 biallelic inactivation（biTP53）as a distinct and independent subtype. Patients within this subtype face an exceedingly grim prognosis with an extremely short survival period. Currently，available treatments for MDS patients with TP53 gene abnormalities have proven ineffective in improving their prognosis. Consequently，there is a growing focus on exploring new avenues such as targeted therapies and immunotherapy. This article provides an in-depth review of the progress in clinical research related to MDS with TP53 gene abnormalities，both within domestic and international contexts.