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Clinical aspects

 

T cell lymphoma in the setting of Sjögren’s syndrome: T cells gone bad? Report of five cases from a single centre cohort


1, 2, 3, 4, 5, 6

 

  1. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece.
  2. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece.
  3. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece.
  4. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece.
  5. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece.
  6. Department of Pathophysiology, School of Medicine National and Kapodistrian University of Athens, Greece. agoules@med.uoa.gr

CER13880
2020 Vol.38, N°4 ,Suppl.126
PI 0125, PF 0129
Clinical aspects

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PMID: 33025901 [PubMed]

Received: 31/07/2020
Accepted : 17/09/2020
In Press: 30/09/2020
Published: 22/10/2020

Abstract

OBJECTIVES:
To identify and record lymphomas of T cell origin in a single centre cohort of 110 Sjögren’s syndrome (SS)-associated non-Hodgkin’s lymphoma (NHL) patients, followed up from 1993 to June 2020.
METHODS:
We searched for patients diagnosed with T cell lymphoma among 110 SS-associated NHL cases. Demographic data, history of previous lymphoma, histologic subtype, lymphoma stage, treatment schedules, and response to therapy were documented.
RESULTS:
Among the 110 SS-associated NHL patients, we identified five NHL cases of T cell origin, all of whom were women. The median time from SS diagnosis to T cell lymphoma development was 3.25 years. They all expressed at least one adverse predictive factor for lymphoma development. Lymphoma subtypes were identified as: two peripheral T cell lymphomas not otherwise specified (NOS) lymphomas, one primary cutaneous T cell lymphoma, one T large granular lymphocyte (T-LGL) leukaemia and one angioimmunoblastic T cell lymphoma. All lymphomas were stage IV, apart from the latter case that was stage III, according to the Ann Arbor staging system. All lymphomas tested positive for T cell receptor (TCR) gamma clonal rearrangements in biopsy specimens, and two were also positive for Epstein-Barr virus-encoded RNA (EBER). Two out of five patients had previously been diagnosed with B cell lymphoma, treated with combined immunochemotherapy, and one had been previously diagnosed with lymph node benign polyclonal follicular hyperplasia.
CONCLUSIONS:
SS-associated T cell lymphomas constitute a minority. Treatment with anti-CD20 monoclonal antibody (mAb) and viral infections may be implicated in their pathogenesis.

Rheumatology Article