Diagnostic dilemma in postpartum associated hemolytic uremic syndrome in a 38th week pregnant 31-year-old Congolese: A case report

Abstract

Background: Thrombotic microangiopathy is associated with HELLP syndrome, thrombotic thrombocytopenic purpura, or atypical hemolytic uremic syndrome (aHUS) during pregnancy. Standard laboratory and physical examinations can help distinguish between these three diseases promptly and guide their management. This is critical because their managements and prognoses differ considerably. The ADAMTS13 test, complement tests, and biopsies can help ascertain the diagnosis; however, they take time, and are not widely available. In this case report, we present a case that highlights the diagnostic and therapeutic dilemmas associated with the aforementioned diseases. Case presentation: A 31-year old P3G3 patient presented at 38 weeks with high blood pressure, bilateral pitting edema, and a low fetal heart rate. A cesarean section was performed to extract the fetus. On postoperative day 2, the suites were marked by anemia, low platelet count, acute kidney injury, declining liver function, and the presence of schistocytes on the peripheral thin smear. The patient was lucid, coherent, and presented no neurological deficits. The ADAMTS13 test and anti-complement therapy were not readily available, so the team made a presumptive diagnosis of aHUS based on the history, clinical presentation, and standard laboratory results. Due to a lack of anticomplement therapy, the patient was prescribed four sessions of hemodialysis. The renal function and platelet count gradually increased, and the patient was discharged on postoperative day 18. The patient was followed for over a year and did not present relapses of thrombocytopenia or microangiopathic hemolytic anemia. Conclusions: The prompt diagnosis and management of aHUS lead to favorable outcomes. Healthcare providers should be able to rapidly differentiate between pregnancy-associated thrombotic microangiopathies and prescribe appropriate management. Here, we highlighted the challenges of diagnosing and managing postpartum associated aHUS in a low-resource setting.