Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials

Blood. 2008 May 1;111(9):4463-70. doi: 10.1182/blood-2007-08-105759. Epub 2008 Feb 21.

Abstract

To evaluate the prognostic significance of clinicobiologic and pathological features in angioimmunoblastic T-cell lymphoma (AITL), 157 AITL patients were retrieved from the GELA LNH87-LNH93 randomized clinical trials. One hundred forty-seven patients received a cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)-like regimen with intensified courses in half of them. Histologically, 41 cases were classified as "rich in large cells" and 116 as "classic" (including 19 rich in epithelioid cells, 14 rich in clear cells, and 4 with hyperplastic germinal centers). Sixty-two cases were scored for CD10 and CXCL13 expression according to the abundance of positive lymphoid cells. Median age was 62 years, with 81% advanced stage, 72% B symptoms, 65% anemia, 50% hypergammaglobulinemia, and 66% elevated LDH. Overall 7-year survival was 30%. In multivariate analysis, only male sex (P = .004), mediastinal lymphadenopathy (P = .041), and anemia (P = .042) adversely affected overall survival. Increase in large cells and high level of CD10 and CXCL13 did not affect survival. Intensive regimen did not improve survival. In conclusion, AITL is a morphologically heterogeneous T-cell lymphoma commonly expressing CXCL13 and CD10 and carrying few prognostic factors. It portends a poor prognosis even when treated intensively. However, AITL is not always lethal with 30% of patients alive at 7 years.

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Chemokine CXCL13 / analysis
  • Cyclophosphamide / therapeutic use
  • Doxorubicin / therapeutic use
  • Humans
  • Immunoblastic Lymphadenopathy
  • Lymphoma, T-Cell / drug therapy
  • Lymphoma, T-Cell / mortality*
  • Lymphoma, T-Cell / pathology*
  • Middle Aged
  • Neprilysin / analysis
  • Prednisone / therapeutic use
  • Prognosis
  • Randomized Controlled Trials as Topic
  • Retrospective Studies
  • Survival Analysis
  • Vincristine / therapeutic use

Substances

  • CXCL13 protein, human
  • Chemokine CXCL13
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Neprilysin
  • Prednisone