Abstract
Background: Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy (TMA)resulting from uncontrolled activation of the complement system during pregnancy or postpartum period. In the intensive care unit, it often becomes necessary to differentiate aHUS from sepsis-related multiple organ dysfunction, thrombotic thrombocytopenic purpura (TTP), and hemolysis, elevated liver enzymes and low platelet (HELLP) syndrome. Early recognition of aHUS is critical for accurate treatment and prognosis. While ADAMTS13 test, peripheral blood broken red blood cell test, complement test, and blood culture are helpful in aHUS diagnosis, these tests are time consuming and may not be widely available. Herein, we present a case of a pregnant woman with severe aHUS during the corona virus disease 2019 (COVID-19) pandemic.
Case presentation: A 26-year-old patient with P4G1(four pregnancies and one delivery) at her 30 weeks and 2 days of pregnancy developed vaginal fluid and showed fetal growth restriction by ultrasound at an external hospital. During labor induction, the patient gradually developed high fever and abnormal coagulation function, followed by heart failure, acute kidney injury, anemia, and severe thrombocytopenia. The patient’s mind was clear and coherent and presented no neurological dysfunction. She was transferred to our department and was provided invasive respiratory support, blood transfusion, continuous renal replacement therapy, capacity management, and other comprehensive treatment. Given the ongoing COVID-19pandemic, ADAMTS13 test and complement blocker were unavailable. The team made a diagnosis of pregnancy-associated aHUS based on history, clinical presentation, and standard laboratory results. The patient was prescribed 13 sessions of hemodialysis. Re-examination after treatment revealed normal complement C3 and C4 levels, stable platelet and hemoglobin levels, and a gradual change in the liver function to normalcy. Her renal function gradually improved, and the bone marrow puncture showed no fragmented red blood cells. The patient was transferred to the department of nephrology on day 40 and back to the local hospital on day 42. The patient was followed over 3 years where she did not show any relapse of thrombocytopenia and microangiopathic hemolytic anemia.
Conclusions: This case reiterates the challenges and importance of diagnosis and treatment of severe pregnancy in a low-resource setting with complex comorbidities, including multiple organ failure and atypical hemolytic uremic syndrome.