OBJECTIVE:To explore the occurrence regularity and clinical characteristics of proteasome inhibitor(PI)related thrombotic microangiopathy(TMA),so as to provide reference for safe drug use in clinic.METHODS:CNKI,Wanfang Data,VIP,PubMed and Web of Science were retrieved to collect case report of PI related TMA up to Apr.30th,2023.General information,medication information,clinical manifestations of TMA,laboratory examination,treatment and prognosis were analyzed.RESULTS:A total of 92 patients in 37 studies were collected,including 46 males(50.00%),29 females(31.52%)and 17 unknown gender(18.48%).The mean age was(61±13)years old.The median time of onset of TMA was 64 d.Main clinical manifestations were fever,fatigue,nausea/vomiting,oliguria/anuria.Due to one patient experiencing TMA twice,a total of 93 cases of TMA occurred,and patients had significantly lower platelet counts,with a median value of 19.5×109/L,with the greatest number of patients having 0 to 50×109/L(52 cases,55.91%).Serum creatinine was elevated in 64 cases(68.82%),with the largest number(32 cases,34.41%)in the range of>177 to 445 μmol/L.After discontinuation and supportive treatment(93 cases),76 cases(81.72%)were recovered/improved,6 cases(6.45%)had sequelae and 5 cases(5.38%)died.The most commonly used drug was carfilzomib(67 cases,72.04%)in PI related TMA.CONCLUSIONS:Attention should be paid to PI related TMA,and factors such as gender and drug categories should be considered.According to clinical manifestations and laboratory examination,adverse drug reactions should be identified as early as possible to ensure the medication safety of patients.