Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lymphoproliferative disorder in renal transplant recipients.
We report 2 cases of renal transplant recipients in whom hepatosplenic gamma-delta T-cell lymphoma (gamma-delta HSTCL) developed 5 and 10 years after transplantation. Both patients had marked hepatosplenomegaly, B symptoms (weight loss, fever, and night sweats), and abnormal peripheral blood findings, including anemia in both, thrombocytopenia and leukoerythroblastic changes in 1, and leukocytosis in the other. Markedly atypical lymphoid infiltrate of intermediate to large cells was observed in the spleen, liver, and bone marrow. The malignant cells showed typical immunophenotype of gamma-delta T cells (CD2+, CD3+, CD4-, CD8-, CD7+, gamma-delta T-cell receptor-positive, and alpha-beta T-cell receptor-negative) with clonal T-cell receptor gene rearrangement and were of the V-delta-1 subset. In addition, the cells contained a cytolytic granule-associated protein, TIA-1, and Fas ligand, indicating cytotoxic T-cell differentiation. The malignant T cells in both cases were of host tissue origin. Both cases were negative for Epstein-Barr virus genome using Southern blot analysis. The patients did not respond to reduction of immunosuppression. Despite initial response to chemotherapy, both patients died within 6 months of diagnosis. Our findings indicate that gamma-delta HSTCL can occur as a late complication in transplant recipients.
Wu
H
H
Department of Pathology, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.
Wasik
M A
MA
Przybylski
G
G
Finan
J
J
Haynes
B
B
Moore
H
H
Leonard
D G
DG
Montone
K T
KT
Naji
A
A
Nowell
P C
PC
Kamoun
M
M
Tomaszewski
J E
JE
Salhany
K E
KE
D000328:Adult; D004273:DNA, Neoplasm / Q000032:analysis; D053222:Fas Ligand Protein; D017809:Fatal Outcome; D005260:Female; D016694:Gene Rearrangement, delta-Chain T-Cell Antigen Receptor / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D016030:Kidney Transplantation / Q000009:adverse effects*; D008113:Liver Neoplasms / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism; D008875:Middle Aged; D039102:Poly(A)-Binding Proteins; D016133:Polymerase Chain Reaction; D011506:Proteins; D016601:RNA-Binding Proteins / Q000378:metabolism; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics / Q000276:immunology*; D015375:Receptors, Interleukin-2 / Q000097:blood; D013160:Splenic Neoplasms / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D000074019:T-Cell Intracellular Antigen-1
D004273:DNA, Neoplasm; C505661:FASLG protein, human; D053222:Fas Ligand Protein; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins; D016692:Receptors, Antigen, T-Cell, gamma-delta; D015375:Receptors, Interleukin-2; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human