Epstein-Barr virus-negative post-transplant lymphoproliferative disorders - A distinct entity?

被引:229
作者
Nelson, BP
Nalesnik, MA
Bahler, DW
Locker, J
Fung, JJ
Swerdlow, SH
机构
[1] Univ Pittsburgh, Sch Med, Dept Pathol, Pittsburgh, PA USA
[2] Univ Pittsburgh, Sch Med, Dept Surg, Pittsburgh, PA USA
[3] Univ S Alabama, Coll Med, Dept Pathol, Mobile, AL 36688 USA
关键词
post-transplant lymphoproliferative disorders; Epstein-Barr virus; lymphoma; transplantation;
D O I
10.1097/00000478-200003000-00006
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Post-transplant lymphoproliferative disorders (PTLDs) are usually but not invariably associated with Epstein-Ban virus (EBV). The reported incidence, however, of EBV-negative PTLDs varies widely, and it is uncertain whether they should be considered analogous to EBV-positive PTLDs and whether they have any distinctive features. Therefore, the EBV status of 133 PTLDs from 80 patients was determined using EBV-encoded small ribonucleic acid (EBER) in situ hybridization stains with or without Southern blot EBV terminal repeat analysis. The morphologic, immunophenotypic, genotypic, and clinical features of the EBV-negative PTLDs were reviewed, and selected features were compared with EBV-positive cases. Twenty-one percent of patients had at least one EBV-negative PTLD (14% of biopsies). The initial EBV-negative PTLDs occurred a median of 50 months post-transplantation compared with 10 months for EBV-positive cases. Although only 2% of PTLDs from before 1991 were EBV negative, 23% of subsequent PTLDs were EBV negative (p <0.001). Of the EBV-negative PTLDs, 67% were of monomorphic type (M-PTLD) compared with 42% of EBV-positive cases (p <0.05). The other EBV-negative PTLDs were of infectious mononucleosis-like, plasma cell-rich (n = 2), small B-cell lymphoid neoplasm, large granular lymphocyte disorder (n = 4) and polymorphic (P) types. B-cell clonality was established in 14 specimens and T-cell clonality was established in three (two patients). None of the remaining specimens were studied with Southern blot analysis and some had no ancillary studies. Rearrangement of c-MYC was identified in two M-PTLDs with small noncleaved-like features, and rearrangement of BCL-2 was found in one large noncleaved-like M-PTLD. Ten patients were alive at 3 to 63 months (only three patients received chemotherapy). Seven patients, all with M-PTLDs, are dead at 0.3 to 6 months. Therefore, EBV-negative PTLDs have distinct features, but some do respond to decreased immunosuppression, similar to EBV-positive cases, suggesting that EBV positivity should not be an absolute criterion for the diagnosis of a PTLD.
引用
收藏
页码:375 / 385
页数:11
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