title: T-cell prolymphocytic leukemia in a 63-year-old female with a pre-existing T-cell large granular lymphocytic leukemia: metachronous T-cell leukemias with discordant subset restrictions (CD4 versus CD8) and distinct clonal identities.
abstract: A 55-year-old female with T-cell large granular lymphocytic leukemia (T-LGL) (CD8+) was initially treated with anti-thymocyte globulin and then cyclosporine due to anemia/neutropenia. While the severity of cytopenia varied with the therapy, the T-LGL persisted. Eight years after the initial diagnosis, she developed lymphadenopathy and hepatosplenomegaly. A complete blood cell count revealed leukocytosis, anemia and thrombocytopenia with ∼ 80% lymphocytes. In contrast to the LGL cells, the blood lymphocytes at this time were medium-large in size and had oval/irregular nuclei, condensed chromatin, indistinct nucleoli and a moderate amount of basophilic cytoplasm, many with elongated vacuoles, and some with cytoplasmic projections. The abnormal lymphocytes comprised ∼ 30% of the bone marrow cellularity with interstitial infiltrates/aggregates. Immunophenotypic analyses demonstrated a T-cell neoplasm with features suggestive of T-cell prolymphocytic leukemia (T-PLL) (CD4+). Cytogenetic analysis revealed a novel clone with complex abnormalities. PCR-based TRG gene rearrangement studies detected a clonal amplicon distinct from that of the preexisting T-LGL. Because of the chronological sequence of the two T-cell neoplasms, this case was initially considered an aggressive transformation of T-LGL. However, this was ultimately excluded by a discordant CD4-subset restriction and the presence of a distinct clonal identity. While these two T-cell neoplasms may have intrinsic connections, the underlying pathogenesis remains to be investigated.
authors.affiliation: Department of Pathology, People's Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi, People's Republic of China; Department of Pathology, Duke University Medical Center, Durham, NC, United States.
authors.forename: Qiang
authors.initials: Q
authors.lastname: Wei
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC, United States.
authors.forename: Paulie
authors.initials: P
authors.lastname: Papavassiliou
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC, United States.
authors.forename: Catherine
authors.initials: C
authors.lastname: Rehder
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC, United States.
authors.forename: Siby
authors.initials: S
authors.lastname: Sebastian
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC, United States. Electronic address: endi.wang@duke.edu.
authors.forename: Endi
authors.initials: E
authors.lastname: Wang
mesh_terms: D016516:CD4-CD8 Ratio / Q000379:methods; D002999:Clone Cells / Q000166:cytology; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D019675:Genes, T-Cell Receptor gamma / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D054066:Leukemia, Large Granular Lymphocytic / Q000235:genetics / Q000473:pathology*; D015461:Leukemia, Prolymphocytic, T-Cell / Q000235:genetics / Q000473:pathology*; D008214:Lymphocytes / Q000473:pathology*; D008875:Middle Aged
keywords: Clonal identities; Metachronous; Subset restrictions; T-cell large granular lymphocytic leukemia; T-cell prolymphocytic leukemia