title: Peripheral T cell lymphoma, not otherwise specified with myelofibrosis: report of a case with review of the literature.
abstract: A 68-year-old man presented to us with pancytopenia, erythroderma, and multiple lymphadenopathies. Lymph node biopsy led to the diagnosis of peripheral T-Cell lymphoma-not otherwise specified (PTCL-NOS). Immunostaining of the lymph node biopsy specimens for cytokines revealed that the tumor cells were positive for plated-derived growth factor (PDGF), basic fibroblast growth factor (b-FGF), vascular endothelial growth factor (VEGF), tumor necrosis factor α (TNF-α), interferon-γ (IFN-γ), interleukin-1β (IL-1β), interleukin-2 (IL-2), and transforming growth factor-β (TGF-β). Bone marrow biopsy revealed infiltration by the PTCL-NOS and myelofibrosis (MF). Bone marrow blood was negative for JAK-2V617F. Bone marrow immunostaining for cytokines showed that the tumor cells were positive for PDGF, b-FGF, VEGF, TNF-α, IFN-γ, IL-1β, IL-2, and TGF-β. The patient was initiated on treatment, and after the first course of CHOP therapy, the bone marrow infiltration by the PTCL-NOS and MF improved. Repeat immunostaining of bone marrow biopsy specimens for cytokines showed that the tumor cells had become negative for PDGF, VEGF, TNF-α and TGF-β. However, after the second course of CHOP therapy, the bone marrow infiltration by the PTCL-NOS and MF worsened. Immunostaining of bone marrow specimens for cytokines again revealed positive staining results of the tumor cells for PDGF, TNF-α, and TGF-β. At the completion of the first course of treatment, the infiltration by the PTCL-NOS improved, but not the pancytopenia.
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Sekiguchi
authors.affiliation: Department of Hematology, Juntendo University Hospital Japan.
authors.forename: Syuichi
authors.initials: S
authors.lastname: Shirane
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Asami
authors.initials: A
authors.lastname: Shimada
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Kunimoto
authors.initials: K
authors.lastname: Ichikawa
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Mutsumi
authors.initials: M
authors.lastname: Wakabayashi
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Keiji
authors.initials: K
authors.lastname: Sugimoto
authors.affiliation: Department of Pathology, Juntendo University Urayasu Hospital Japan.
authors.forename: Shigeki
authors.initials: S
authors.lastname: Tomita
authors.affiliation: Department of Pathology, Juntendo University Urayasu Hospital Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Izumi
authors.affiliation: Department of Clinical Laboratory, Juntendo University Urayasu Hospital Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Nakamura
authors.affiliation: Department of Clinical Laboratory, Juntendo University Urayasu Hospital Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Sawada
authors.affiliation: Department of Pathology, Research Hospital, Institute of Medical Science, University of Tokyo Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ohta
authors.affiliation: Department of Hematology, Juntendo University Hospital Japan.
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Noguchi
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000097:blood; D001706:Biopsy; D001853:Bone Marrow / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D001856:Bone Marrow Examination; D003520:Cyclophosphamide / Q000627:therapeutic use; D016207:Cytokines / Q000097:blood; D004317:Doxorubicin / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000097:blood / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000097:blood / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014408:Biomarkers, Tumor; D016207:Cytokines; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone
keywords: Peripheral T-Cell lymphoma-not otherwise specified (PTCL-NOS); bone marrow fibrosis (MF); platelet-derived growth factor (PDGF); transforming growth factor-β (TGF-β); tumor necrosis factor α (TNFα)