Peripheral T cell lymphoma, not otherwise specified with myelofibrosis: report of a case with review of the literature

Int J Clin Exp Pathol. 2015 Apr 1;8(4):4186-203. eCollection 2015.

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.

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).

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / blood
  • Biopsy
  • Bone Marrow Examination
  • Bone Marrow* / drug effects
  • Bone Marrow* / immunology
  • Bone Marrow* / pathology
  • Cyclophosphamide / therapeutic use
  • Cytokines / blood
  • Doxorubicin / therapeutic use
  • Humans
  • Immunohistochemistry
  • Lymph Nodes* / drug effects
  • Lymph Nodes* / immunology
  • Lymph Nodes* / pathology
  • Lymphoma, T-Cell, Peripheral* / blood
  • Lymphoma, T-Cell, Peripheral* / drug therapy
  • Lymphoma, T-Cell, Peripheral* / immunology
  • Lymphoma, T-Cell, Peripheral* / pathology
  • Male
  • Prednisolone / therapeutic use
  • Primary Myelofibrosis* / blood
  • Primary Myelofibrosis* / drug therapy
  • Primary Myelofibrosis* / immunology
  • Primary Myelofibrosis* / pathology
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Vincristine / therapeutic use

Substances

  • Biomarkers, Tumor
  • Cytokines
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Prednisolone

Supplementary concepts

  • VAP-cyclo protocol