From the archives of MD Anderson Cancer Center: Untreated leukemic non-nodal mantle cell lymphoma with relapse as pleomorphic variant mantle cell lymphoma 21 years later

Ann Diagn Pathol. 2021 Feb:50:151649. doi: 10.1016/j.anndiagpath.2020.151649. Epub 2020 Oct 24.


Leukemic, non-nodal mantle cell lymphoma (MCL) is a distinct, rare, indolent variant of mantle cell lymphoma, but can relapse aggressively. It can present with lymphocytosis with chronic lymphocytic leukemia (CLL)-like morphologic and immunophenotypic features as was initially considered in the index case. However, at time of splenectomy, two years later cyclin D1 overexpression was shown and the disease was realized to be leukemic non-nodal MCL. The patient was followed for 21 years, without therapy, before he developed clinically aggressive MCL with lymphadenopathy. Lymph node biopsy showed MCL, pleomorphic variant. We review the literature and discuss the features of leukemic non-nodal MCL as well as the potential pitfalls in diagnosis. Furthermore, we are not aware of another cases reported with a 21 year interval from initial diagnosis of leukemic non-nodal MCL to aggressive MCL.

Keywords: Indolent; Latency; Leukemic; Mantle cell lymphoma; Pleomorphic.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biopsy
  • Cyclin D1 / metabolism*
  • Follow-Up Studies
  • Humans
  • Immunophenotyping / methods
  • Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis
  • Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology*
  • Lymphadenopathy / pathology
  • Lymphocytosis / etiology
  • Lymphocytosis / pathology
  • Lymphoma, Mantle-Cell / diagnosis
  • Lymphoma, Mantle-Cell / metabolism
  • Lymphoma, Mantle-Cell / pathology*
  • Lymphoma, Mantle-Cell / therapy
  • Lymphoma, Non-Hodgkin / pathology*
  • Male
  • Recurrence
  • Remission Induction
  • Splenectomy / methods
  • Stem Cell Transplantation / methods
  • Transplantation, Homologous / methods


  • CCND1 protein, human
  • Cyclin D1