Transformation in Low-grade B-cell Neoplasms

Surg Pathol Clin. 2016 Mar;9(1):79-92. doi: 10.1016/j.path.2015.09.004.

Abstract

Low-grade B-cell leukemias/lymphomas are a diverse group of indolent lymphoproliferative disorders that are typically characterized by good patient outcomes and long life expectancies. A subset of cases, however, undergo histologic transformation to a higher-grade neoplasm, a transition associated with a more aggressive clinical course and poor survival. Transformation of follicular lymphoma to diffuse large B-cell lymphoma and Richter transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma are best characterized in the literature. This article reviews clinical and pathologic characteristics of these most common forms of transformation, with an emphasis on salient histologic, immunophenotypic, and genetic features.

Keywords: CLL/SLL; Clonality; Follicular lymphoma; Low-grade lymphoma; Richter syndrome; Transformation.

Publication types

  • Review

MeSH terms

  • Cell Transformation, Neoplastic / pathology
  • Diagnosis, Differential
  • Disease Progression
  • Humans
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology*
  • Lymphoma, Follicular / diagnosis
  • Lymphoma, Follicular / pathology*
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Neoplasm Grading