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Jankowska, I.1; Paw owska, J.13; Kaliciñski, P.2; Teisseyre, M.1; Czubkowski, P.1; Gliwicz, D.1; Dura, W.3; Kamiñski, A.2; Markiewicz, M.2; Socha, J.1
1Gastroenterology, Hepatology and Immunology, 2Pediatric Surgery and Organ Transplatation, 3Pathology, Children’s Memorial Health Institute, Warsaw, Poland
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Introduction: Posttransplant lymphoproliferative disease (PTLD) in children remains a significant clinical problem.
Methods: 156 pediatric liver transplantations were performed between March 1990 and December 2002 in Children’s Memorial Health Institute. Five of the children aged 18–29 months experienced EBV-associated PTLD. Recipients’ age at the time of transplantation ranged from 12 to 17 months. PTLD was diagnosed within 3 to 13 months after transplantation. The immunosuppressive regimen of all patients with PTLD consisted of prednisone and tacrolimus. Four pts had EBV VCA IgG before Ltx.
Results: The following signs and symptoms were observed: anemia (5), fever (4), hypoalbuminemia (3), anorexia (2), chronic diarrhea (3), values of EBV PCR genomes/105 lymphocytes ranged 400–5000 /N300 – University of Pittsburgh/ (5), biopsies of the lymph nodes revealed two types of histology: plasmacytic hyperplasia (2) and polymorphic B-cell infiltration (3).
Patients were treated with immunosuppression withdrawal (4) or reduction (1) and intravenous antiviral therapy. In one case anti-CD20 monoclonal antibody (rituximab) was given intravenously eight weeks after the onset of the disease. Clinical improvement and EBV-PCR reduction were observed in all patients. All children developed acute liver graft rejection episodes, confirmed by liver histology - tacrolimus therapy was then reintroduced. One patient had reccurence of PTLD 34 months after the first episode. The duration of follow-up after the onset of PTLD is 9–39 months. All children are alive.
Conclusion: PTLD is an important complication of immunosuppressive therapy, usually encountered in younger children after LTx.
In case of non-specific signs/symptoms further PTLD tests should be considered.
Reduction of immunosuppression is an effective therapy but must be administered cautiously regarding the acute rejection risk.
© 2004 Lippincott Williams & Wilkins, Inc.
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