Initial therapy of mantle cell lymphoma

David J. Inwards, Thomas E. Witzig

Research output: Contribution to journalReview article

3 Scopus citations

Abstract

Mantle cell lymphoma is a well-recognized distinct clinicopathologic subtype of B-cell non-Hodgkin lymphoma. The current World Health Organization (WHO) classification subdivides this entity into aggressive and other variants. The disease has a predilection for older males, and patients typically present at an advanced stage with frequent splenomegaly and extranodal involvement including bone marrow, peripheral blood, gastrointestinal, and occasional central nervous system involvement. Early studies of therapy outcomes in this disease revealed that while response rates where high, relapse was expected after a limited period of time. Prolonged survival was uncommon, with initial median survival rates typically in the 3–4-year range. Those with a high proliferative rate, blastoid morphology, and selected clinical features were recognized as having a worse prognosis. Therapeutic approaches have diverged into aggressive therapies with high response rates and promising progression free survival rates, which may be applied to younger healthy patients, and less aggressive approaches. Aggressive therapies include intensive chemotherapy alone or chemotherapy followed by autologous stem cell transplant, which has been shown to be most effective when applied in first remission. Whether these more intense therapies result in improved survival as compared with less aggressive therapies is not well established. Allogeneic transplant has also been investigated, although high treatment-related mortality and the risk of chronic graft versus host disease and the relatively advanced age of this patient population have tempered enthusiasm for this approach. A number of less aggressive therapies have been shown to produce promising results. Consolidation and maintenance strategies are an active area of investigation. A number of newer agents have shown promising activity in relapsed disease, and are being investigated in the front-line setting. Overall survival rates are improving in this disease, with current studies suggesting a median survival of 5 or more years.

Original languageEnglish (US)
Pages (from-to)381-392
Number of pages12
JournalTherapeutic Advances in Hematology
Volume2
Issue number6
DOIs
StatePublished - Dec 2011

Keywords

  • chemotherapy
  • mantle cell lymphoma
  • stem cell transplant

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'Initial therapy of mantle cell lymphoma'. Together they form a unique fingerprint.

  • Cite this