Diffuse large B-cell non-Hodgkin's lymphoma in a patient with autoimmune lymphoproliferative syndrome

Br J Haematol. 2001 May;113(2):432-4. doi: 10.1046/j.1365-2141.2001.02749.x.

Abstract

Mutations of Fas or Fas ligand genes result in the autoimmune lymphoproliferative syndrome (ALPS) in humans. We report here a diffuse large B-cell non-Hodgkin's lymphoma occurring in a man with ALPS. Fas-mediated lymphocyte apoptosis was defective in vitro, owing to a mutation within the death domain of the Fas molecule. High-dose methotrexate and doxorubicin-based chemotherapy led to complete remission of lymphoma.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antigens, CD20 / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Apoptosis
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • Axilla
  • Biomarkers / analysis
  • Bone Marrow / immunology
  • Humans
  • Immunohistochemistry
  • Immunosuppressive Agents / therapeutic use
  • Lymph Nodes / immunology
  • Lymphoma, B-Cell / complications*
  • Lymphoma, B-Cell / drug therapy
  • Lymphoma, B-Cell / immunology
  • Lymphoproliferative Disorders / complications*
  • Lymphoproliferative Disorders / drug therapy
  • Lymphoproliferative Disorders / immunology
  • Male
  • Methotrexate / therapeutic use
  • Mutagenesis, Insertional
  • Neuropeptides / genetics
  • Polymerase Chain Reaction
  • Receptors, Tumor Necrosis Factor*
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / pathology
  • fas Receptor / pharmacology

Substances

  • Antigens, CD20
  • Biomarkers
  • FAS protein, human
  • Immunosuppressive Agents
  • Neuropeptides
  • Receptors, Tumor Necrosis Factor
  • fas Receptor
  • Methotrexate