Clonal and morphological variation in a posttransplant lymphoproliferative disorder: evolution from clonal T-cell to clonal B-cell predominance.
The majority of posttransplant lymphoproliferative disorders (PTLD) are Epstein-Barr virus (EBV)-associated and of B-cell origin. A much smaller proportion of PTLD are of T-cell origin. We report the clinical, morphological, immunophenotypic, and genotypic results of a unique PTLD, initially diagnosed as immune mediated thrombocytopenia (ITP), which at presentation was predominantly an anaplastic appearing EBV-associated T-cell PTLD and, after reduction in immunosuppression and the administration of antiviral agents, predominantly an EBV-associated plasma cell rich B-cell PTLD. Subsequent chemotherapy resulted in a complete remission. This case has both practical and biological implications. It highlights how PTLD may be misdiagnosed as other entities, how biclonal cases can have different morphological appearances and include both B- and T-cell clones, how PTLD can evolve over time possibly related to immune reconstitution, and why PTLD should be rebiopsied when the disease does not respond to decreased immunosuppression or recurs.
Nelson
B P
BP
University of South Alabama College of Medicine, Mobile, USA.
Locker
J
J
Nalesnik
M A
MA
Fung
J J
JJ
Swerdlow
S H
SH
D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D001854:Bone Marrow Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology / Q000821:virology; D017252:CD3 Complex / Q000378:metabolism; D002999:Clone Cells / Q000378:metabolism / Q000473:pathology*; D019674:Genes, T-Cell Receptor beta / Q000235:genetics; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D007146:Immunoglobulin lambda-Chains / Q000378:metabolism; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D016031:Liver Transplantation / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000209:etiology / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology; D012367:RNA, Viral / Q000032:analysis / Q000378:metabolism; D013154:Spleen / Q000276:immunology* / Q000378:metabolism / Q000473:pathology / Q000821:virology; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology* / Q000821:virology
D017252:CD3 Complex; C099537:Epstein-Barr virus encoded RNA 1; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D012367:RNA, Viral