Expression of S100 Protein in CD4-positive T-cell Lymphomas Is Often Associated With T-cell Prolymphocytic Leukemia

• Published in: The American journal of surgical pathology Vol. 39; no. 12; p. 1679
• Main Authors: Aggarwal, Nidhi, Pongpruttipan, Tawatchai, Patel, Snehal, Bayerl, Michael G, Alkan, Serhan, Nathwani, Bharat, Surti, Urvashi, Kitahara, Sumire, Chinthammitr, Yingyong, Swerdlow, Steven H
• Format: Journal Article
• Language: English
• Published: United States 01.12.2015
• Subjects: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor - analysis; Biomarkers, Tumor - genetics; Biopsy; CD4-Positive T-Lymphocytes - chemistry; CD4-Positive T-Lymphocytes - immunology; Child; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Kaplan-Meier Estimate; Leukemia, Prolymphocytic, T-Cell - genetics; Leukemia, Prolymphocytic, T-Cell - immunology; Leukemia, Prolymphocytic, T-Cell - metabolism; Leukemia, Prolymphocytic, T-Cell - mortality; Leukemia, Prolymphocytic, T-Cell - pathology; Lymphoma, T-Cell - chemistry; Lymphoma, T-Cell - genetics; Lymphoma, T-Cell - immunology; Lymphoma, T-Cell - mortality; Lymphoma, T-Cell - pathology; Male; Middle Aged; Predictive Value of Tests; Prognosis; S100 Proteins - analysis
• ISSN: 1532-0979
• DOI: 10.1097/PAS.0000000000000496

S100 T-cell lymphomas are infrequent, and except 1 all have been CD4 negative. On the basis of an index case of CD4 S100 T-cell prolymphocytic leukemia (T-PLL), we studied S100 protein expression in 19 additional T-PLLs and 56 other T-cell lymphomas that are usually CD4, including 15 angioimmunoblastic T-cell lymphomas, 24 anaplastic large cell lymphomas (16 ALK and 8 ALK), 7 mycosis fungoides/Sézary syndrome, and 10 peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS). Two additional S100 CD4 PTCL, NOS cases were also reviewed. Thirty percent (6/20) of T-PLLs were S100 compared with 0/56 other T-cell lymphomas with previously unstudied S100 reactivity (40 CD4, 2 CD8, 11 CD4/CD8, 3 unknown) (P=0.0007). There were no significant differences between the S100 and S100 T-PLLs with regard to the male:female ratio (2:1 vs. 1:1), age (71.6±7.7 vs. 65.4±9.3), peripheral blood lymphocyte count (67.2±116.6 vs. 101.1±159.7×10/L), or median survival (463 vs. 578 d, where known). The 2 S100 PTCL, NOS cases occurred in a 7-year-old boy and a 45-year-old woman. Both had involvement of the bone marrow and peripheral blood but were morphologically unlike T-PLL and lacked TCL1 gene rearrangement. These results demonstrate that S100 T-cell lymphomas include a subset that are CD4 and most often, but not exclusively, are T-PLL. Although having diagnostic implications, there were no documented clinical differences between the S100 and S100 T-PLLs.