Real-time, Multidisciplinary Approach of Women With Pregnancy-Associated Thrombotic Microangiopathy.

INTRODUCTION: Thrombotic microangiopathies (TMAs) occurring during pregnancy or the immediate postpartum period are a rare, and highly complex syndrome. Determination of the most appropriate therapeutic strategy remains challenging and requires individualized assessment.

METHODS: To support both diagnostic and therapeutic decision-making, a national multidisciplinary consultation committee [MCC] (titled "TMA and Pregnancy") was established in October 2023 under the auspices of the French National Reference Center for TMAs. The MCC is activated for a single case, on an emergency basis, and within 24 hours. It brings together national experts from 8 medical specialties.

RESULTS: Here, we report on 2 years of experience of the MCC, including operational aspects, activity indicators, the clinical and obstetrical profiles of the first 76 women whose cases were reviewed, the suspected diagnoses, and the real-time impact on patient management. Of the women, 29 (38%) were primiparous. The obstetrical context was preeclampsia in 47% and peripartum hemorrhage (PPH) in 40% of the women. TMA was most frequently attributed to severe endotheliosis, with acute kidney injury (AKI) present in 47 women (62%), including 9 (12%) showing renal cortical necrosis (RCN). Thrombotic thrombocytopenic purpura (TTP) was relatively uncommon (n = 13, 17%), and no cases were considered to represent atypical hemolytic and uremic syndrome (aHUS). The MCC led to a change in management in 26% of cases, most often a deescalation of therapy.

CONCLUSION: We emphasize the value of real-time multidisciplinary case conferences in managing catastrophic obstetrical syndromes and advocate for the recognition of extreme phenotypes of endotheliosis extending beyond the liver. These findings underscore the need for therapies targeting endothelial injury.