Blood Journal
Leading the way in experimental and clinical research in hematology

Brief report
IDH2 mutations are frequent in angioimmunoblastic T-cell lymphoma

  1. Rob A. Cairns1,*,
  2. Javeed Iqbal2,*,
  3. François Lemonnier3,*,
  4. Can Kucuk2,
  5. Laurence de Leval4,
  6. Jean-Philippe Jais5,
  7. Marie Parrens6,
  8. Antoine Martin7,
  9. Luc Xerri8,
  10. Pierre Brousset9,
  11. Li Chong Chan10,
  12. Wing-Chung Chan2,
  13. Philippe Gaulard3, and
  14. Tak W. Mak1
  1. 1Campbell Family Institute for Breast Cancer Research at Princess Margaret Hospital, University Health Network, Toronto, ON;
  2. 2Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE;
  3. 3Université Paris-Est, Inserm U955, Department of Pathology, Hôpital Henri Mondor, Créteil, France;
  4. 4Institute of Pathology, Centre Hospitalier Universitaire (CHU) Vandois University Hospital of Lausanne, Lausanne, Switzerland;
  5. 5Université Paris-Descartes, Assistance Publique–Hopitaux de Paris (AP-HP), Hôpital Necker, Service de Biostatistique, Paris, France;
  6. 6Département de Pathologie, Hôpital du Haut l'Évêque, Pessac, France;
  7. 7Department of Pathology, AP-HP, CHU de Bobigny, Bobigny, France;
  8. 8Department of Biopathology, Institut Paoli-Calmettes, Marseille, France;
  9. 9Department of Pathology, University Paul Sabatier Toulouse III and Cancer Research Center of Toulouse, Unité Mixte de Recherche 1037, CHU Purpan, Toulouse, France; and
  10. 10Department of Pathology, University of Hong Kong, Hong Kong


Mutations in isocitrate dehydrogenase 1 (IDH1) and isocitrate dehydrogenase 2 (IDH2) occur in most grade 2 and 3 gliomas, secondary glioblastomas, and a subset of acute myelogenous leukemias but have not been detected in other tumor types. The mutations occur at specific arginine residues and result in the acquisition of a novel enzymatic activity that converts 2-oxoglutarate to D-2-hydroxyglutarate. This study reports IDH1 and IDH2 genotyping results from a set of lymphomas, which included a large set of peripheral T-cell lymphomas. IDH2 mutations were identified in approximately 20% of angioimmunoblastic T-cell lymphomas (AITLs), but not in other peripheral T-cell lymphoma entities. These results were confirmed in an independent set of AITL patients, where the IDH2 mutation rate was approximately 45%. This is the second common genetic lesion identified in AITL after TET2 and extends the number of neoplastic diseases where IDH1 and IDH2 mutations may play a role.

  • Submitted November 11, 2011.
  • Accepted December 21, 2011.
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