ESPN 51th Annual Meeting

ESPN 2018


 
Renal transplant and Glanzmann thrombasthenia: peri-operative management of a patient at very high hematological risk and immunization
PAUL BASTARD 1 ANNE COUDERC 1 THERESA KWON 1 JULIEN HOGAN 1 OLIVIER NIEL 1 LIZA ALI 2 ANNE MAISIN 1 GEORGES DESCHENES 1 MARIE-FRANCOISE HURTAUD 3 MARIE-ALICE MACHER 1

1- PEDIATRIC NEPHROLOGY UNIT, ROBERT DEBRE HOSPITAL, AP-HP, PARIS, FRANCE
2- PEDIATRIC SURGERY UNIT, ROBERT DEBRE HOSPITAL, AP-HP, PARIS, FRANCE
3- CRC MALADIES HEMORRAGIQUES CONSTITUTIONNELLES, SERVICE DHEMATOLOGIE BIOLOGIQUE, CHU ROBERT DEBRE, AP-HP, PARIS, FRANCE
 
Introduction:

The hemorrhagic risk for kidney transplantation (KTX) is considerably higher when there is platelet dysfunction. We report the case of a 12 years old boy with type 2 Glanzmann thrombasthenia (GT) who underwent successful living donor kidney transplantation (LDKTX).

Material and methods:

This patient started peritoneal dialysis (PD) in Algeria at the age of 1 year. GT was diagnosed at 9 years old, after hemorrhagic shock following circumcision and change of PD catheter. Numerous blood and platelets transfusions (PT) were needed. As a result, he was hyper-immunized. He was referred in our center for KTX in 2016.

Results:

After meetings with hemostasis specialists, LDKTX appeared mandatory to permit the convocation of compatible platelet donors and to make the monitoring of the preventive treatment of hemorrhagic risk. The father had positive CDC crossmatch, thus the mother was selected as donor because of a negative FC crossmatch, despite the presence donor-specific antibodies (DSA). ABO and HLA compatible platelet donors were identified and convened to donate for the surgery and after. Tranexamic acid was given orally the day before surgery, followed by intravenous infusion on the day of surgery, and then oral treatment was reinitiated for 10 days post-operatively. Prophylactic ABO and HLA compatible PT were made every 8 to 12h during the first 48h. Following surgery, graft function was normal and no severe bleeding was noted. After week 5, several episodes of macroscopic hematuria occurred, with obstruction and anuria: the J-J ureteric stent was replaced 4 times in 2 months. Finally, macroscopic hematuria stopped. The ureteric stent was removed without complication 9 months after TX. At month 18 (presently), creatininemia is at 67 µmol/L and the patient has no detectable DSA.

Conclusions:

To our knowledge, this is the first report of a successful LDKTX strategy, with multidisciplinary management, in a patient with type 2 GT.