Retrospective analysis of mortality and morbidity among thrombotic thrombocytopenia purpura presenting to Georgia cancer center

Abstract Introduction: Thrombotic thrombocytopenia purpura (TTP) is a serious blood disorder with mortalities reaching 90% in patients who are left untreated and as high as 20% those who are treated with plasmic exchange (PLEX), immunosuppression, and immunotherapy. TTP requires a multidisciplinary team including a team of hematologists, pathologists, and critical care physicians generally at a tertiary medical center which presents a challenge for patients presenting through medical centers not equipped to treat such conditions. The purpose of this study was to analyze mortality and morbidity of patients requiring transfer to a primary apheresis center. Methods: This was a retrospective cohort study analyzing patients admitted to a primary apheresis center from 2016-2022. Patients aged 18 or older with PLASMIC scores greater than 4 were presumed to have TTP. Characteristics analyzed included age, distance to primary aphesis center (<100 miles or >100 miles), initial PLASMIC score, time for initiation of PLEX (<10 hours or >10 hours), initial LDH and creatine levels, and recovery of platelets (plt count day 1 vs day 3) and neurological symptoms(10 hours before or after the initiation of PLEX). Results: A total of 22 patients with PLASMIC score greater than 4 (average 6) were analyzed. Later confirmation of TTP was made with ADAMTS13 activity levels less than 10% noted in 18 of 22 patients (81%). Of the 22 patients, 4 expired (18%) of which only 1 patient presented from an outside facility greater than 100 miles away. The primary outcome of time to PLEX did not appear to have a significant outcome of mortality as the average time of initiation of PLEX for patients who expired was 10.5 hours compared to 17.7 hours in patients who survived (p value 0.10). Secondary outcomes showed nonsignificant difference in length of stay of patients receiving PLEX 10 hours prior versus after presentation (p value 0.40). Similar outcomes were appreciated in resolution of neurological symptoms (p value 0.74) and recovery of platelet count at day 3 (p value 0.34). However, there was a significant correlation of elevated creatine at baseline leading to increase length of stay (p value 0.01). Conclusions: TTP is a life-threatening condition that is not treated early and approximately can lead to high mortality rates. While results of the study showed nonsignificant data, this can be explained by low statistical power as well as confounding factors including severity of TTP at presentation leading to increase mortality and length of stay. More data collection and analysis is needed to further examine mortality and morbidity of patients presenting to a primary apheresis center versus a non-apheresis center. The further analysis may lead to future guidelines improving mortality and morbidity in patients presenting to a non-apheresis medical center.