Successful Multivisceral plus Kidney Transplantation of a highly sensitised paediatric

recipient; with Eculizumab salvage for hyperacute renal rejection

King’s College HospitalNHS Trust

NHS

Warner S, Heaton N, Vilca-Melendez H, Vaughn R, Taylor J, Drage M, Thompson R, Hind JM

Liver, Gastrointestinal and Nutrition Centre, King's College Hospital, London, United Kingdom

BACKGROUND

•Patients with preformed donor specific antibodies (DSA), are at high risk of developing antibody mediated rejection (AMR) in small bowel and kidney transplantation

•Eculizumab has been used for the treatment of AMR in kidney transplantation but there is no reported use in paediatric intestinal transplant recipients

We present the case of a 13 year old girl withProgressive Familial Intrahepatic Cholestasis type I, 10 years after primary liver transplantation, but with high DSA

HISTORY

The patient received a liver transplant for end-stage chronic liver disease at age 3. Over the next 10 years suffered from severe diarrhoeal episodes, leading to frequent dehydration and hospitalisations. Ultimately intestinal failure developed.

Internal then external biliary diversion failed to improve the intestinal function. Total external biliary diversion was subsequently performed to remove all bile acids from the gut with short-term improvement. Within weeks intestinal failure deteriorated and persisted despite feed modification including modular triglyceride-free feed. During this period the episodes of severe dehydration together with recurrent sepsis led to deterioration in the liver graft and renal function. Liver cirrhosis and chronic renal failure ensued.

•Multivisceral plus kidney transplantation was performed though the patient had a DSA MFI of 17,557, with Class II anti-HLA antibodies DQ.

•Induction desensitisation was with plasma exchange and Alemtuzumab.

•On day 2 a diagnosis of hyperacute AMR renal rejection was made

•

OUTCOME

•Eculizumab was used as salvage therapy. – Creatinine returned to normal.– DSA levels fell– DSA negative one month post-transplant– DSA negligible since (10 months follow-up)

Now 10 months post multivisceral transplant she continues to have good graft function in all organs

ECULIZUMAB

•Humanized monoclonal antibody•Specifically binds to complement protein C5•Prevents cleavage to activated forms C5a and C5b. •The complement cascade is stalled and cannot form the membrane attack complex C5b-9.

CONCLUSION

Successful multivisceral transplantation can be achieved in highly sensitised paediatric recipients with planning of post transplant immunosuppression and use of novel agents such as Eculizumab

REFERENCES

•Am J Transplant. 2015 Feb 3. doi: 10.1111/ajt.13183. [Epub ahead of print]. Eculizumab Salvage Therapy for Antibody-Mediated Rejection in a Desensitization-Resistant Intestinal Re-Transplant Patient.

Fan J et al.

 

•Pediatrics 2015 Feb;135(2):e551-5. doi: 10.1542/peds.2014-2275.

Eculizumab to treat antibody-mediated rejection in a 7-year-old kidney transplant recipient.

Chehada H et al.

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