A clinicopathological study of nodal marginal zone B-cell lymphoma. A report on 21 cases

Histopathology. 2006 Jan;48(2):162-73. doi: 10.1111/j.1365-2559.2005.02309.x.


Aims: To report the clinicopathological findings of 21 cases of primary nodal marginal zone B-cell lymphoma (NMZL). NMZL is a recently characterized lymphoma and few series have been published.

Methods and results: The clinical data were characteristic of a disseminated disease at presentation: presence of peripheral and abdominal lymph nodes, bone marrow involvement (62%), disease stage III and IV (76%), elevated lactate dehydrogenase (LDH) (48%). Other features included peripheral blood involvement (23%), anaemia (24%), thrombocytopenia (10%) and presence of serum M component (33%), while the previously reported association with hepatitis C virus and cryoglobulinaemia was not found. Relapses were frequent but the majority of patients receiving chemotherapy had a good initial response. Morphological features were heterogeneous and there were some differences compared with other marginal zone B-cell lymphomas (MZL). Pure monocytoid B-cell lymphomas were rare (10%) but a minor component of monocytoid B cell was observed more frequently (23%). Plasmacytoid or plasmacytic differentiation was a very common feature (61%). Large cells and a high mitotic count were also frequent (57%).

Conclusion: NMZL can be distinguished from splenic MZL and extranodal MZL by its aggressive morphology and disseminated disease at presentation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD20 / analysis
  • Bone Marrow / pathology
  • DNA-Binding Proteins / analysis
  • Female
  • Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics
  • Humans
  • Immunohistochemistry
  • Karyotyping
  • Lymph Nodes / pathology
  • Lymphoma, B-Cell / genetics
  • Lymphoma, B-Cell / metabolism
  • Lymphoma, B-Cell / pathology*
  • Male
  • Middle Aged
  • Neoplasm Staging
  • Proto-Oncogene Proteins c-bcl-2 / analysis
  • Proto-Oncogene Proteins c-bcl-6
  • Survival Analysis
  • Translocation, Genetic


  • Antigens, CD20
  • BCL6 protein, human
  • DNA-Binding Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • Proto-Oncogene Proteins c-bcl-6