POS-724 THROMBOTIC MICROANGIOPATHY AND EBV RELATED POST-TRANSPLANT LYMPHOPROLIFERATIVE DISORDER IN A YOUNG PATIENT: A CASE REPORT

Abstract

Post-transplant lymphoproliferative disorders (PTLD) are recognized as a devastating complication of organ transplantation. In kidney transplants the incidence is estimated between 1 and 2%. They have been closely associated with Epstein-Barr virus (EBV), and the spectrum of manifestations after transplantation range from an infectious mononucleosis-like syndrome to more serious polyclonal or monoclonal lymphoproliferation. Herein we report a case of an EBV associated PLTD in a young patient A 12 years old boy received a paternal renal transplant for kidney failure secondary to Ig A nephropathy in december 2012. They both had old immunity (IgG positif and IgM negatif) to EBV. They had 3 mismatchs and the induction treatment was based on basiliximab, ciclosporin and steroids , the maintenance therapy consisted of ciclosporine, prednisone and MMF Post tranplant complications were marked by the onset of a VZV infection after 2 months which responded well to aciclovir treatment On october 2013, he was admitted to the transplant unit for exploration of an alteration in graft function and thrombopenia ; initlaly he was treated as an acute rejection by solumedrol boli and a biopsy of the graft was performed. The C2 dosage of ciclosporin returned very high being in the zone of toxicity and the result of the biopsy confirming it with the diagnosis of a thrombotic microangiopathy (TMA) secondary to anti calcineurins (ciclosporin) After 1 month , the patient presented episodes of rectal bleeding with gastrointestinal fibroscopy and left colonsocpy returned to normal with fever, earaches and elevated LDH without alteration of the graft’s function A specialized examination was performed objectifying a bilateral sero mucous otitis and we completed with a pelvic thoraco abdomino and cavum CT scan which showed multiple cervical and inguinal lymphadenopathy with nodular splenomegaly and the EVB pcr came back positive We completed with a fine needle aspiration and lymph node biospy diagnosing a lymph node localisation of diffuse large B cell lymphoma . Initial management consisted on withdrawl of immunosuppression and the prescription the chimeric anti-CD20 monoclonal antibody rituximab and transfusion of platelet pellets but the patient presented a severe thrombocytopenia with alteration of neurological condition secondary to meningeal haemorrhage and the result was fatal. Although the induction treatment in our case was based on basiliximab and the patient was Ig G positif to EBV before transplatation , he presented a severe form of an EBV related PLTD.