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Short report
Large granular lymphocyte expansions in primary Sjögren’s syndrome: characteristics and outcomes
  1. Alistair Baber1,
  2. Gaetane Nocturne1,2,
  3. Roman Krzysiek3,
  4. Julien Henry1,
  5. Rakiba Belkhir1,
  6. Xavier Mariette1,2 and
  7. Raphaele Seror1,2
  1. 1Department of Rheumatology, AP-HP, Paris-Sud University Hospitals, Le Kremlin Bicêtre Hospital, Le Kremlin-Bicêtre, France
  2. 2INSERM U1184, Center for Immunology of Viral Infections and Autoimmune Diseases, Paris-Sud University, Paris-Saclay University, Le Kremlin-Bicêtre, France
  3. 3Department of Biologic Immunolgy, AP-HP, Paris-Sud University Hospitals, Le Kremlin Bicêtre Hospital, Le Kremlin-Bicêtre, France
  1. Correspondence to Dr Raphaele Seror; raphaele.seror{at}aphp.fr

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Key messages

What is already known about this subject?

  • Large granular lymphocyte expansion (LGLe) have frequently been described in association with rheumatoid arthritis, but rarely with primary Sjögren’s syndrome (pSS).

  • The role of LGLe in neutropenia following treatment with rituximab (RTX) has already been suggested in patients with lymphoma.

What does this study add?

  • Our study suggests for the first time that LGLe associated neutropenia could be a side effect of RTX in patients with pSS.

  • Methotrexate could be useful in case of severe neutropenia.

How might this impact on clinical practice?

  • RTX being more and more used in systemic auto-immune diseases, this possible effect must be known.

Primary Sjögren’s syndrome (pSS) is a systemic auto-immune disease (AID) that mainly causes mucosal dryness, asthenia, arthralgia and, sometimes, severe organ involvement requiring immunosuppressive treatment. Despite negative randomised controlled trials, rituximab (RTX) is frequently used in case of systemic complications.1

Large granular lymphocyte (LGL) disorders are characterised by proliferation of LGL cytotoxic lymphocytes of either T-cell (mainly CD3+TCRαβ+CD8+CD57+CD56+/−CD16+/−, rarely CD4+CD8+/−) (T-LGL) or less frequently NK-cell (CD3-CD2+CD16+CD56+CD57+/−) (NK-LGL) origin. The spectrum of LGL disorders extends from reactive LGL expansions (LGLe), which are usually asymptomatic, transitory and have polyclonal or oligoclonal profile, to LGL leukaemias, which are monoclonal proliferations associated with cytopenias and somatic activating mutations in STAT3.2 T-LGL expansions (T-LGLe) have frequently been described in several AIDs,2 especially rheumatoid arthritis (RA). In this context, they could exhibit either polyclonal, oligoclonal or monoclonal profile, and could be accompanied by neutropenia irrespective of their clonal status.3 However, LGLe have been exceptionally reported in pSS. Thus, we here report eight cases of LGLe in patients with pSS.

In our study, all patients from the Paris-Sud hospital, national reference centre for pSS, diagnosed with pSS and having a LGLe were retrospectively included between September 2016 and March 2018. Diagnosis of pSS was based on ACR/EULAR 2016 criteria.4 Three of our eight patients did …

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