P2511 - A Case of Atypical Hemolytic Uremic Syndrome in an Elderly Woman

Ogonna Ngwu, MD, David Murillo-Garcia, MD
Mercy Health, Cincinnati, OH

Introduction: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by a triad of hemolytic anemia, acute kidney injury and thrombocytopenia. HUS typically affects the kidneys but has extra-renal manifestations including the gastrointestinal, neurological cardiac systems. Atypical HUS is a rare disorder with a prevalence of 2.21 to 9.4 per million people aged 20 years or younger, children between the ages of 0 and 4 are most affected¹. This disorder is rare among the elderly.

Case Description/Methods: A 78-year-old woman presented to the emergency department with complaints of fatigue and back pain associated with nausea, and vomiting for one week. Emergency department workup revealed urinary tract infection, elevated creatinine, leukocytosis, and low platelets. She was transferred to our center. The patient reported taking diclofenac as needed for osteoarthritis.Physical examination revealed an ill-appearing elderly female with dry, tacky mucous membranes, delayed capillary refill, and suprapubic tenderness. Laboratory testing showed schistocytes on peripheral blood smear, LDH was high, haptoglobin was low, shiga toxin was negative. ADAMSTS 13 activity was ~ 35%, renal biopsy showed evidence of thrombotic microamgiopathy (TMA)/fibrin deposition within arterioles confirming diagnosis. During hospitalization, the patient had melanotic stools as well as an episode of altered mentation and decreased responsiveness. CT head did not show any acute abnormality. Abdominal ultrasound revealed new nodular contour of the liver without splenomegaly. Renal ultrasound was normal. EGD revealed a 6 cm sliding hiatal hernia, large 2 cm ulcer with flat pigmented spot in the gastric antrum, no cameron ulcers and no esophageal varices.The patient was started on eculizumab and given pneumococcal, meningococcal vaccination and penicillin prophylaxis. Patient required hemodialysis. She responded well to eculizumab with resolution of thrombocytopenia, hemolytic anemia, and neurologic symptoms. She was discharged on weekly eculizumab with plan to change to ravulizumab infusion 8 weekly. She unfortunately was still requiring hemodialysis at the time of discharge.

Discussion:




This case shows the importance of early diagnosis and initiating eculizumab early. HUS is rare in adults, and a high index of suspicion is needed for diagnosis in uncommon populations. Management also involves early consultation of oncology, nephrology as multidisciplinary team approach is required.


Disclosures:


Ogonna Ngwu, MD, David Murillo-Garcia, MD. P2511 - A Case of Atypical Hemolytic Uremic Syndrome in an Elderly Woman, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.