Simultaneous phenotypically distinct but clonally identical mucosa- associated lymphoid tissue and follicular lymphoma in a patient with Sjogren's syndrome

Antonella Aiello, Ming Qing Du, Tim C. Diss, Huai Zheng Peng, Francesco Pezzella, Daniela Papini, Roberto Giardini, Silvana Pilotti, Lang Xing Pan, Peter G. Isaacson

Research output: Contribution to journalArticlepeer-review

Abstract

A 44-year-old woman with a 12-year history of Sjogren's syndrome (SS) developed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma in the parotid gland. Two years later, she presented with generalized lymphadenopathy and hepatosplenomegaly and a follicular lymphoma was diagnosed. To investigate the relationship of the two histologically distinct lymphomas, we re-examined their histology and immunophenotype and studied the lymphomatous tissue from the parotid, cervical lymph node, and spleen using molecular genetic methods. Histologic and immunophenotypic studies confirmed the previous diagnoses and also identified a previously unnoticed focus of follicular lymphoma in the second parotid gland biopsy. Polymerase chain reaction (PCR) amplification of the rearranged Ig heavy-chain gene showed the same sized dominant product in the MALT lymphoma and the follicular lymphoma. Similarly, PCR analysis of the t(14:18) translocation yielded an identical sized band from both MALT and follicular lymphoma. Cloning and sequencing of the Ig PCR products showed an identical CDR3 sequence from each lesion, indicating a common clonal lineage. The follicular lymphoma of the parotid gland lymph node and the follicular lymphoma of the spleen showed an identical mutation signature to that of the salivary gland MALT lymphoma. We propose that follicular lymphoma in the parotid gland lymph node may have resulted from colonization of lymphoid follicles by MALT lymphoma cells, following which the tumor cells were induced to express a follicular lymphoma phenotype, due to Bcl-2 overexpression caused by t(14;18), leading to a change in clinical behavior resulting in rapid widespread dissemination of disease. These observations suggest that the distinct phenotypes of low-grade B-cell lymphomas may be the consequence of interplay between genetic and local microenvironmental factors.

Original languageEnglish
Pages (from-to)2247-2251
Number of pages5
JournalBlood
Volume94
Issue number7
Publication statusPublished - Oct 1 1999

ASJC Scopus subject areas

  • Hematology

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