Real-time, multidisciplinary approach of women with pregnancy-associated thrombotic microangiopathy

Introduction Thrombotic microangiopathies (TMA) occurring during pregnancy or in the immediate postpartum period are a rare, and highly complex syndrome. The 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 Thrombotic Microangiopathies. Its originality stands in that this committee is activated for one single case, on an emergency basis, and within 24 hours. It brings together national experts from eight medical specialties. Results Here, we report two years of experience of this committee, 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. The majority of women were primiparous (n=29, 38%). The obstetrical context was a preeclampsia in 47% of women, peri-partum hemorrhage in 40%. TMA was most frequently attributed to severe endotheliosis, with acute kidney injury present in 47 (62%) of women, including 9 (12%) showing renal cortical necrosis. Thrombotic thrombocytopenic purpura was relatively uncommon (n=13, 17%), and no cases were considered to represent a first episode of atypical HUS. The MCC led to a change in management in 26% of cases, most often a de-escalation of therapy. Conclusions We emphasize the value of real-time multidisciplinary case conferences (MCCs) 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.