AbstractAimWe reviewed case reports of acute fatty liver of pregnancy (AFLP) in Japan and summarized its characteristics, including coagulation factors, fibrinolytic factors, and platelet counts.MethodsPubMed/Medline and Ichushi databases from 2000 to 2022 were used to survey articles related to AFLP, and 93 articles (102 patients) were ultimately identified.ResultsThe characteristics of the coagulation and fibrinolysis systems in AFLP were as follows: the prothrombin time‐international normalized ratio and activated partial prothrombin time were prolonged (median value [quartiles]: 1.59 [1.31, 2.02] and 47.5 s [28.2, 97.5], respectively), and antithrombin and alpha 2‐antiplasmin levels were low (23.0% [17.0, 33.0] and 44.6%, respectively), indicating non‐productive coagulopathy due to severe liver damage. The concentrations of the thrombin‐antithrombin complex and fibrinogen/fibrin degradation products were high (60.0 ng/mL [49.1, 82.8] and 49.2 μg/mL [20.8, 143.7], respectively), while the level of fibrinogen was low (82.0 mg/dL [52.5, 153.5]), suggesting disseminated intravascular coagulation with increased coagulation activity. A platelet count ≥12 × 104/μL was detected in approximately 70% of AFLP cases, and the median was within the normal range (16.1 × 104/μL [11.1, 19.2]).ConclusionsCoagulopathy changes in AFLP may be based on non‐productive coagulopathy of coagulation and fibrinolytic factors as well as disseminated intravascular coagulation with mild or no thrombocytopenia.