Thrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are often mistaken for preeclampsia or HELLP. Unfortunately, delays in appropriate diagnosis and treatment may be life-threatening. Our objective is to alert obstetrician-gynecologists, certified nurse midwives, family medicine providers, and subspecialty consultants, to the range of TMA disorders that may occur in and around pregnancy. To do this, we have provided a review of individual disorders that comprise the differential diagnosis of pregnancy TMA, and we have proposed a systematic approach to make an accurate diagnosis with readily available clinical and laboratory data. In complex or critical cases, we recommend a multidisciplinary team approach (e.g., Critical Care, Hematology, Maternal Fetal Medicine, Nephrology) to expedite diagnosis and treatment, which may be life-saving.
Keywords: Atypical hemolytic uremic syndrome; HELLP (hemolysis, Elevated liver enzymes, Low platelet count syndrome); Preeclampsia with severe features; Pregnancy; Thrombotic microangiopathy.
Copyright © 2018 International Society for the Study of Hypertension in Pregnancy. Published by Elsevier B.V. All rights reserved.