From may 1996 to september 2001 2/11 (1.8%) of our heart lung transplanted children and 1/37 (0.03%) of our heart transplanted children developed PTLD (tissue-proven in 2 cases) 32.3 months (6, 4 and 87 months after transplantation).

Patient demographics: mean age at time of transplantation 6.2 years, 1 male, 2 females, diagnosis: pulmonary fibrosis and primary pulmonary hypertension (patient 1 and 2) in the 2 heart lung transplanted patients and right heart failure after Senning procedure for TGA in the heart transplanted patient (patient 3). All 3 patients had negative EBV serology before transplantation. EBV seroconversion after transplantation occured in all 3 patients. There was serologic evidence of EBV infection at the time of PTLD diagnosis. EBV could be detected in PTLD tissue biopsies of 2/3 patients.

Cyclosporine (CsA) was the primary immunosuppressive agent in the 3 patients. One heart lung transplanted patient had acute rejection of the lung (treated with high dose steroids) shortly after transplantation and was converted from CsA to tacrolimus (Tac). He developed PTLD 6 months afterwards. The other heart lung transplanted patient had 2 acute pulmonary rejections (treated with high dose steroids) and developed a recidive of PTLD one year afterwards.

Patient 1 (HLTx) who developed lymphoproliferative disease had substantial reduction of axillar lymph node enlargement after conversion from Tac to CsA. Patient 2 (HLTx) with PTLD histologically diagnosed in lymphoadenoid tissue and bowel and recurrent PTLD histologically detected in lung tissue was treated with reduction of immunotherapy and chemotherapy. Patient 3 (HTx) with histologically proven PTLD in a submandibular lymph node was treated with reduction of immunosuppression and chemotherapy. All 3 patients still show positive EBV virus loads detected by PCR.