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A typical Hemolytic Uremic Syndrome Trigger Lead to Renal Allograft Failure

Akbar Mahmood

After a week of commercial renal transplant, recipient reported to emergency without any details of donor recipient workup, HLA typing, cross matching & induction therapy or surgery, Underwent through a stormy course of events. Atypical haemolytic uremic syndrome (aHUS), acute cellular rejection (ACR), grafts failure despite aggressive management, with rATG and eculizumab.

Asymptomatic, adequate urine output. PMHx: Was on hemodialysis (permacath) for one year. Renal failure was due to thrombotic microangiopathy (TMA) aHUS based on biopsy& molecular testing.  Eculizumab started but she lost follow up till she appeared A&E post kidney transplant. BMI=36,BP 160/100mmHg. Surgical wound dehiscence (right iliac fossa) with pus discharge, (swab negative). Serum creatinine 115 mmol/l with low phosphate level. Hb 11.4 g/dl.

Based on previous renal disease, suspicion of TMA was postulated. Renal allograft biopsy was performed. Consistent with borderline ACR and TMA. ACR  treatment initiated with methyl- prednisolone and rATG (6 mg/kg total dose). Creatinine continued worsening with rising LDH. Blood films showed moderate schistocytes consistent with aHUS. aHUS screen and ADAMTS13 ordered. Eculizumab started [3], had meningitis vaccine. Flexible cystoscopy and DJ stent removed. 2nd week: Fever spike with burning micturition. UTI suspected with Infectious disease opinion vancomycin and meropenem started empirically along with oral fluconazole. Immunossuppression continued. Carbapenem resistant enterococci colonization of the central line (removed). Gallium scan was negative for any infective focus. Colistin was added for multidrug resistant (MDR) bacteremia. Meropenem continued for 10 days and voriconazole for 1 month. Clinical improvement observed with resolution of sepsis. HRCT chest, bronchoscopy revealed no endobronchial lesion seen. Bronchoalveolar lavage (BAL) for gram stain, culture, viral PCRs and PJP turned out to be negative. Cr continued to deteriorate (peaked to 361 on day 46 of transplant). US Doppler showed no intra-renal blood flow due to thrombus. After multi-disciplinary team meeting (MDT) with nephrology, radiology, vascular surgery and urology, conventional renal angiography was decided in view to possible thrombolysis in an attempt to salvage. Risks explained to the patient and family and MDT recommendations. Informed consent obtained. Right iliac angiogram showed complete thrombosis of right internal iliac and renal artery (as shown). 24h Thrombolytic infusion did not show revascularization MDT concluded allograft failure due to aHUS Right jugular permacath placed, hemodialysis.