[1466] ΒF1 Negative, T-Cell Receptor Gamma Chain Constant Region Positive (TCRGC, Clone γ3.20) Intestinal T-Cell Lymphomas

AL Wilson, SH Swerdlow, U Surti, J Choi, RE Felgar. University of Pittsburgh, Pittsburgh, PA; Children's Hospital, Philadelphia, PA

Background: Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumor of cytotoxic T-cells, often associated with celiac disease, unique HLA haplotypes and recurrent genetic abnormalities including 9q amplification. The WHO definition includes 2 types, without specific exclusion of a γδ origin. However, it has been suggested that γδ cases should be classified as mucocutaneous γδ T-cell lymphoma.
Design: Clinical, gross, microscopic, flow cytometric (FC) and/or paraffin immunohistochemical phenotype, and EBER staining of 5 small intestine T-cell lymphomas, βF1-negative, with positivity for TCRGC by paraffin IHC (clone γ3.20, Thermo Scientific, Rockford, IL, AJCP 2009;13:820-6), were evaluated, including 1 definite TCR-γδ+ case (FC). 9q31.3 amplification was assessed by FISH (c-abl probe, Vysis).
Results: Patients had no known skin, spleen or other mucocutaneous involvement. 2 had adenopathy on CT. 3/3 had negative BM. 1 had lung involvement 10 mos after diagnosis (only survivor).4/5 patients died of disease (0-10 mos, med 7). All showed transmural invasion, 3 perforated, and a monomorphic infiltrate of intermediate to large cells, with ulceration and extensive mucosal infiltration overlying tumor, and no reactive inflammatory infiltrate. All were CD3, CD7, TIA-1, and TCRGC positive and CD4, CD5, CD25, PD-1, FOXP3, βF1, CD30, ALK-1, and EBER negative, some staining for CD2 (3/5) and granzyme B (1/5). 1 had confirmed TCR-γδ and weak CD103 by FC. Intra-epithelial lymphocytes (IELs) away from tumor were CD3+, TIA-1+, CD8+, with negative CD5 and CD30 in 3 cases evaluated. 4/5 showed 9q polysomies, including case 1.

Features of TCRGC+, Beta F1- Intestinal Lymphomas
Case No/Age/SexKnown EnteropathyMultifocal Intestinal DiseaseVillous Atrophy (Away from Tumor)IELs (Away from Tumor)CD56CD8TCRγδ (FC)
#1 / 31 / MNoYesYesYes+-+
#2 / 63 / MYes (58 yrs)NoYesYes-+Equivocal
#3 / 90 / FNoYesNoYes+-N/A
#4 / 72 / MNoYesNoYes+-N/A
#5 / 73 / FNoNoYesYes--N/A
N/A = Not assessed.


Conclusions: While γδ T-cell lymphomas of other sites have distinct features from EATL, βF1-, but TCRGC+ (IHC) intestinal lymphomas show overlap with type II EATL (monomorphic medium-sized cells), but with a phenotype overlapping with type I EATL and +9q abnormalities in a confirmed γδ T-cell case. 4 of our TCRGC+ cases may not be of true γδ T-cell origin, as a small subset of other βF1+ T-cell lymphomas may be TCRGC+ (4/28 tested). (J Choi, unpub).
Category: Hematopathology

Monday, March 22, 2010 9:30 AM

Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 196, Monday Morning

 

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