First- time use of plasma exchange for atypical hemolytic uremic syndrome in a 5-year-old child in a low-middle-income country: a case report

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, caused by dysregulation of the alternative complement pathway. We report the first documented case of aHUS managed with plasma exchange (PLEX) in Bhutan. A previously healthy 5-year-old girl presented with fever, hematuria, severe anemia, thrombocytopenia, and renal dysfunction. Secondary causes were excluded and she was diagnosed with aHUS. Due to unavailability of complement inhibitors, she was managed with corticosteroids, peritoneal dialysis, transfusions, and delayed initiation of PLEX. Following PLEX, hematologic parameters and urine output improved, allowing dialysis discontinuation after 24 days. Renal biopsy revealed focal cortical necrosis with partial renal recovery. She remained in intensive care for 27 days, followed by transfer to the pediatric ward and discharge four days later. At two-month follow-up, she remained dialysis-independent with stable but impaired renal function, highlighting management challenges in resource-limited settings.