Abstract: TH-PO626

HUStling for HELLP: A Case of Pregnancy Induced Atypical HUS (aHUS) and HELLP Syndrome

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Khan, Huda N., Methodist Dallas Medical Center, Dallas, Texas, United States
  • Collazo-Maldonado, Roberto L., Methodist Dallas Medical Center, Dallas, Texas, United States
  • Sebastian, Lisa M., Methodist Dallas Medical Center, Dallas, Texas, United States
Background

Atypical or complement mediated Hemolytic uremic syndrome (aHUS) is a rare disorder mostly associated to gene mutations of complement factors and/or antibodies to complement factors. In rare cases it may be triggered by the stress of pregnancy. This is a case of a pregnant woman with aHUS that initially presented with HELLP Syndrome which made the diagnosis of aHUS challenging.

Methods

A 32 y/o AA woman G6P4A1 with no known past medical history presented to the ER at 25 weeks of gestation with acute onset of lower abdominal pain that started 48 hours prior to admission. On her physical exam, she was very hypertensive at 180/100 mmHg. Labs revealed new onset of proteinuria (urine prot/creat 9.5 g), elevated AST 226/ALT 48, thrombocytopenia 83,000, anemia Hgb 8.5/ Hct 24.9. She was admitted with initial diagnosis of pre-eclampsia with HELLP. She was taken to L&D due to abruptio placenta and intrauterine fetal demise. D&C was performed which was complicated with massive blood loss requiring multiple blood transfusions. She developed anuric AKI that required CRRT. Her condition stabilized, but remained anuric and was still dependent on intermittent dialysis. She continued to be hypertensive and developed anemia with schistocytes on the peripheral smear. Renal biopsy revealed thrombotic microangiopathy and classic findings of aHUS. ADAMTS13 level was normal. Patient was treated with plasmapheresis for 7 days followed by Eculizumab. Once treatment was initiated, the patient’s condition improved with resolution of AKI and hemolysis, and normalization of platelet count. She was discharged to continue indefinite treatment with Eculizimab as outpatient.

Conclusion

Complement mediated HUS may present as a complication from pregnancy. The diagnosis of aHUS during pregnancy may be challenging because the clinical presentation may resemble that of HELLP syndrome as both conditions can coexist and may present with similar laboratory findings. Nephrologists should have high level of suspicion for aHUS in a pregnant woman who presents with HELLP Syndrome. Renal biopsy is valuable in making appropriate diagnosis.