title: Expression of the promyelocytic leukemia zinc-finger in T-lymphoblastic lymphoma and leukemia has strong implications for their cellular origin and greater association with initial bone marrow involvement.
abstract: The promyelocytic leukemia zinc-finger (PLZF) is essential for the development of innate T cells (as represented by natural killer T cells) for acquisition of their unique innate immune properties. We evaluated the PLZF protein expression in a variety of immature and mature lymphoid malignancies. PLZF was preferentially expressed in T-lymphoblastic lymphoma/acute lymphoblastic leukemia (T-LBL/ALL) in 50% of the 54 cases. Among 51 cases of peripheral T-cell lymphoma not otherwise specified, only one (2%) expressed PLZF. One mycosis fungoides case expressed PLZF in lymph node involved by tumor. Otherwise, PLZF was not detected in any other type of lymphoma. In T-LBL/ALL, PLZF expression was more common in CD4/CD8 double-negative (67%) or CD8 single-positive subtypes (73%) than in CD4/CD8 double-positive (13%) and CD4 single-positive subtypes (0%) (P=0.001). Importantly, PLZF and CD1a expression were mutually exclusive in T-LBL/ALL (P=0.001). This was also the case for T-cell receptor βF1 expression (P=0.000). Most (96%) of the PLZF-positive T-LBL/ALL cases showed initial bone marrow involvement compared with 39% of PLZF-negative cases (P=0.000). Based on these findings, we suggest that T-LBL/ALLs that express PLZF arise from early immature double-negative thymocytes when the T-cell receptor β chain has not yet expressed or innate T-cell precursors, and strongly imply bone marrow involvement.
authors.affiliation: Department of Pathology, Seoul National University Hospital, Seoul, South Korea. junarplus@chol.com
authors.forename: Yoon K
authors.initials: YK
authors.lastname: Jeon
authors.forename: Heounjeong
authors.initials: H
authors.lastname: Go
authors.forename: Soo J
authors.initials: SJ
authors.lastname: Nam
authors.forename: Bhumsuk
authors.initials: B
authors.lastname: Keam
authors.forename: Tae M
authors.initials: TM
authors.lastname: Kim
authors.forename: Kyeong C
authors.initials: KC
authors.lastname: Jung
authors.forename: Hyoung J
authors.initials: HJ
authors.lastname: Kang
authors.forename: Dong S
authors.initials: DS
authors.lastname: Lee
authors.forename: Joo R
authors.initials: JR
authors.lastname: Huh
authors.forename: Seong H
authors.initials: SH
authors.lastname: Park
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D001853:Bone Marrow / Q000473:pathology*; D019070:Cell Lineage; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D051741:Kruppel-Like Transcription Factors / Q000378:metabolism*; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000378:metabolism / Q000473:pathology; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000378:metabolism* / Q000473:pathology; D000075722:Promyelocytic Leukemia Zinc Finger Protein; D060168:Thymocytes / Q000378:metabolism / Q000473:pathology; D055815:Young Adult; D016335:Zinc Fingers
chemical_list: D014408:Biomarkers, Tumor; D051741:Kruppel-Like Transcription Factors; D000075722:Promyelocytic Leukemia Zinc Finger Protein; C092037:ZBTB16 protein, human