Early onset, EBV- PTLD in pediatric liver-small bowel transplantation recipients

A spectrum of plasma cell neoplasms with favorable prognosis

Anamarija M. Perry, Patricia Aoun, Donald W Coulter, Warren G. Sanger, Wendy J. Grant, Peter F. Coccia

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

EBV- posttransplantation lymphoproliferative disorders (PTLDs) are rare compared with EBV+ PTLDs, occur later after transplantation, and have a poor response to treatment. Few studies have reported EBV - PTLD in pediatric solid-organ transplantation recipients. We describe 5 cases of EBV- PTLD in recipients of combined liver and small bowel allografts ranging in age from 16 months to 7 years. EBV-PTLD developed 9-22 months (median, 15) after transplantation. Morphologically, the lesions ranged from atypical plasma cell hyperplasia (a term not currently included in the World Health Organization classification) to plasmacytoma like. In all cases, in situ hybridization for EBV was negative, and molecular studies demonstrated clonal IgH gene rearrangements. Protein electrophoresis showed multiple clonal paraproteins in 4 of 5 cases. In 2 cases with a donor-recipient sex mismatch, FISH cytogenetics demonstrated that the plasma cells were of mixed donor/recipient origin. One patient died before therapy. Four patients were treated with high-dose dexamethasone, and 1 patient subsequently required thalidomide. All 4 remain in remission 75-128 months (median, 86) after diagnosis. In contrast to reports of EBV-PTLD in adults, these plasma cell lesions occurred early after transplantation and resolved completely after minimal treatment.

Original languageEnglish (US)
Pages (from-to)1377-1383
Number of pages7
JournalBlood
Volume121
Issue number8
DOIs
StatePublished - Feb 21 2013

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Plasma Cell Neoplasms
Pediatrics
Lymphoproliferative Disorders
Human Herpesvirus 4
Liver
Transplantation
Plasmas
Transplantation (surgical)
Paraproteins
Plasma Cells
Thalidomide
Electrophoresis
Dexamethasone
Allografts
Genes
Health
Tissue Donors
Plasmacytoma
Gene Rearrangement
Organ Transplantation

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Early onset, EBV- PTLD in pediatric liver-small bowel transplantation recipients : A spectrum of plasma cell neoplasms with favorable prognosis. / Perry, Anamarija M.; Aoun, Patricia; Coulter, Donald W; Sanger, Warren G.; Grant, Wendy J.; Coccia, Peter F.

In: Blood, Vol. 121, No. 8, 21.02.2013, p. 1377-1383.

Research output: Contribution to journalArticle

Perry, Anamarija M. ; Aoun, Patricia ; Coulter, Donald W ; Sanger, Warren G. ; Grant, Wendy J. ; Coccia, Peter F. / Early onset, EBV- PTLD in pediatric liver-small bowel transplantation recipients : A spectrum of plasma cell neoplasms with favorable prognosis. In: Blood. 2013 ; Vol. 121, No. 8. pp. 1377-1383.
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