Allegheny General Hospital, USA
Scientific Tracks Abstracts: J Blood Disord Transfus
Introduction: Atypical hemolytic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA). It involves dysregulation of the alternate complement pathway affecting multiple organ systems. Here, we present a previously unreported case of aHUS developing after post-ERCP pancreatitis. Case report: A 52-year-old female with a history of cholelithiasis presented with abdominal pain and jaundice. Physical exam revealed RUQ tenderness without guarding. Laboratory findings showed normal cell counts with elevated bilirubin and liver enzymes. MRCP revealed gallstones with biliary sludge and she underwent ERCP with sphincterotomy. Post-ERCP, she developed severe epigastric pain with vomiting and worsening jaundice. Labs revealed elevated lipase consistent with ERCP induced pancreatitis. After four days, she developed acute anemia, thrombocytopenia and acute renal failure requiring hemodialysis. Laboratory data showed elevated bilirubin, LDH and low Haptoglobin levels. Kidney biopsy confirmed thrombotic microangiopathy. Atypical HUS was diagnosed based on low ADAMTS 13 activity, thrombocytopenia and hemolytic anemia in the presence of pancreatic and renal dysfunction. Therapy with eculizumab was initiated and laboratory findings after 10-weeks of eculizumab therapy showed improvement of platelet count, anemia and renal recovery. Conclusion: Differentiating aHUS from other causes of TMA is challenging, though ADAMTS 13 activity and stool testing can exclude TTP and HUS from aHUS. The aHUS needs to be accurately diagnosed since different pathology will necessitate different therapies. Prompt diagnosis and treatment of aHUS is very essential as early initiation of therapy with eculizumab has a major impact on survival and preservation of renal function.
Suresh Manapuram has received his MBBS degree from Rangaraya Medical College affiliated to NTR University of Health Sciences, India. He has then served for Ministry of Health, Government of India for 2 years as a Medical Officer in Primary Health Care Center. He has also pursued a 2 year Clinical Teaching and Research Fellowship Program at St. George’s University, Grenada. He has served as a Faculty Member in the Department of Neurosciences and Physiology for 3 years at Xavier University School of Medicine, Aruba. He has then joined Allegheny Health Network in July 2014; currently he is a Resident in Internal Medicine Residency Program at Allegheny General Hospital, Pittsburgh. His career interests include Academic Medicine, Hematology and Oncology.
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