title: Donor-Derived T-Cell Large Granular Lymphocytic Leukemia in a Patient With Peripheral T-Cell Lymphoma.
abstract: T-cell large granular lymphocytic (T-LGL) leukemia after hematopoietic stem cell transplantation (SCT) is rare and its natural history and clinical outcome have not been well described. We report the clinical, morphologic, immunophenotypic, and molecular features of a case of donor-derived T-LGL leukemia in a 16-year-old man who received allogeneic SCT for peripheral T-cell lymphoma not otherwise specified (PTCL-NOS). The patient presented with persistent neutropenia and splenomegaly 9 months after SCT when the chimerism study showed a 100% donor pattern. A splenectomy revealed T-LGL leukemia. Flow cytometric analysis showed an aberrant T-cell population positive for CD3, CD5 (dim, subset), CD7, CD8, CD16 (subset), CD57, CD94 (dim, partial), and T-cell receptor (TCR) αβ, and negative for CD4, CD26, CD56, and TCRγδ. Molecular studies showed monoclonal TCRβ and TCRγ gene rearrangements. Both the immunophenotype and molecular profile of the T-LGL leukemia were different from the pre-SCT PTCL. Sequencing analysis for STAT3 exon 21 did not reveal any mutation in both pre-SCT and post-SCT specimens. The patient did not receive any treatment for T-LGL leukemia; however, his count progressively increased after splenectomy, despite the presence of persistent T-LGL leukemia in the bone marrow. There was no evidence of recurrent PTCL. We propose an algorithm to diagnose this rare post-SCT neoplasm.
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Juliana E
authors: JE
authors: Hidalgo Lopez
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Mariko
authors: M
authors: Yabe
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Adrian A
authors: AA
authors: Carballo-Zarate
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Sa A
authors: SA
authors: Wang
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Jeffrey L
authors: JL
authors: Jorgensen
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Sairah
authors: S
authors: Ahmed
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: John
authors: J
authors: Lee
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Shaoying
authors: S
authors: Li
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Ellen
authors: E
authors: Schlette
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Timothy
authors: T
authors: McDonnell
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Roberto N
authors: RN
authors: Miranda
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: L Jeffrey
authors: LJ
authors: Medeiros
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: Carlos E
authors: CE
authors: Bueso-Ramos
authors: From the Departments of Hematopathology and Stem Cell Transplantation, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors: C Cameron
authors: CC
authors: Yin
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015415:Biomarkers; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003131:Combined Modality Therapy; D015321:Gene Rearrangement; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis* / Q000209:etiology*; D008198:Lymph Nodes / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D016609:Neoplasms, Second Primary; D010641:Phenotype; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D014019:Tissue Donors; D014184:Transplantation, Homologous
chemical_list: D015415:Biomarkers; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta