EBV‐negative lymphoproliferative disease with hyper‐IgA, in a child with combined liver and small bowel transplantation

:  A 4‐year‐old boy presented 14 months after liver and small bowel transplantation with fever, diarrhea, elevated liver enzymes, thrombocytopenia and autoantibodies. Total gammaglobulins level was normal but the level of plasma IgA1 was very high. The blood PCR for Epstein–Barr virus (EBV) was nega...

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Bibliographic Details
Published in:Pediatric transplantation 2004-06, Vol.8 (3), p.305-307
Main Authors: Robert, Clotilde Des, Lacaille, Florence, Canioni, Danielle, Quartier‐dit‐Maire, Pierre, Talbotec, Cécile, Goulet, Olivier
Format: Article
Language:English
Subjects:
Autoantibodies - blood
Biological and medical sciences
Child, Preschool
Epstein-Barr virus
Fundamental and applied biological sciences. Psychology
Fundamental immunology
General aspects
Herpesvirus 4, Human - isolation & purification
Humans
immunoglobulin A
Immunoglobulin A - blood
intestinal transplantation
Intestine, Small - pathology
Intestine, Small - transplantation
Liver Transplantation - adverse effects
Lymphoproliferative Disorders - diagnosis
Lymphoproliferative Disorders - etiology
Lymphoproliferative Disorders - immunology
Lymphoproliferative Disorders - virology
Male
Medical sciences
Plasma Cells - pathology
post‐transplant lymphoproliferative disease
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Transplantation, Homologous - adverse effects
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Summary::  A 4‐year‐old boy presented 14 months after liver and small bowel transplantation with fever, diarrhea, elevated liver enzymes, thrombocytopenia and autoantibodies. Total gammaglobulins level was normal but the level of plasma IgA1 was very high. The blood PCR for Epstein–Barr virus (EBV) was negative. The ileal biopsy disclosed a lymphoplasmacytic infiltration. The EBER probe was negative on the small bowel biopsies. The child was considered as suffering from a non‐EBV‐induced posttransplant lymphoproliferative disorder (PTLD). The high IgA level was presumed to be secreted by proliferating plasma cells in the transplanted bowel. Immunosuppression was reduced; but the efficacy was incomplete and an anti‐CD20 antibody was added. There was complete resolution of symptoms and normalization of the IgA level. As IgA1 is mostly of intestinal origin, this unusual presentation of PTLD should lead to a high suspicion of a small bowel proliferating process.
ISSN:1397-3142
1399-3046
DOI:10.1111/j.1399-3046.2004.00174.x