M GANGADHARAN KOMALA1, E FISCHER1, K SUD1,2, B BOSE1,2
1Department of Renal Medicine, Nepean Hospital, Kingswood, New South Wales; 2University of Sydney – Nepean Clinical School, Sydney, New South Wales
Background: Gemcitabine induced hemolytic uraemic syndrome (GiHUS) is rare and can occur in 0.02 to 2.2% of all patients administered this drug. We report a case of GiHUS resistant to corticosteroids and plasma exchange (PEX) that was successfully treated with Eculizumab.
Case Report: A 55 years old female presented with shortness of breath 3 weeks after her last dose of gemcitabine given for ampullary carcinoma. She was found to have acute kidney injury with a creatinine of 125 umol/L and was anemic with a hemoglobin of 66 g/L. She had evidence of microangiopathic hemolytic anemia (MAHA), and also had 7.54 g/day of proteinuria. A kidney biopsy revealed features consistent with thrombotic microangiopathy. She had normal left ventricular (LV) function on echocardiogram and PET scan did not show evidence of active malignancy.
She was diagnosed with GiHUS and commenced on prednisolone 55 mg daily and received PEX. However her renal functions deteriorated with peak creatinine of 297 umol/L. She developed progressive LV dysfunction with a nadir ejection fraction (EF) of 20% and needed to commence hemodialysis for management diuretic resistant pulmonary edema. PEX was ceased after 5 treatments due to poor clinical improvement, persistent MAHA with a normal ADAMTS13 activity of 99% and she was commenced on Eculizumab, with which the hemolytic markers normalised and renal function improved over two months. She is currently dialysis independent and LV function has improved to an EF of >30%.
Conclusion: GiHUS is a rare entity associated with severe morbidity and mortality. Eculizumab should be considered in patients with persistent HUS after cessation of gemcitabine. LV dysfunction associated with aHUS is an increasingly recognized complication that also improves with Eculizumab.