Date: Monday, October 22, 2018
Session Title: Sjögren's Syndrome – Basic and Clinical Science Poster
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Large granular lymphocyte (LGL) proliferations can be observed in some auto-immune disorders, especially rheumatoid arthritis. However, reports of LGL proliferations in primary Sjogren’s syndrome (pSS) are extremely rare. We here described the main characteristics and outcome of 9 patients presenting LGL proliferation in the context of pSS.
All patients from the reference centre for systemic autoimmune diseases (in particular for pSS) diagnosed with pSS (ACR/EULAR 2016 criteria) and having a LGL proliferation were included. LGL proliferations were characterized by blood lymphocyte immunophonetyping and their TCR clonality by PCR. Clinical data and outcome were analyzed for all patients.
Nine pSS patients (8 women; median age of 67 [range 52 to 87] years) were diagnosed with LGL proliferation. All but one had active disease at the time of LGL diagnosis (median ESSDAI 11 [range 6 to 35]). Two patients had a history of lymphoma, one developed LGL proliferation under chemotherapy and the other in the context of an indolent and untreated lymphoma. In 8/9 patients, LGL proliferations were T-CD8 (T-LGL), and only one was of NK-type (NK-LGL). LGL proliferation was monoclonal in 4/9 and oligoclonal in 4/9 (data missing for patient with NK-LGL proliferation). A STAT3 mutation was searched in 2 patients and was negative in both. Neutropenia was the main reason for searching for a LGL proliferation ; neutrophil count was < 1500/mm3 in 7/9 and < 500/mm3 in 3/9 patients. At diagnosis, median lymphocyte cell count was 1120/mm3 (range 480 to 7250) and median LGL cell count was 440/mm3 (range 150 to 6000) representing 23% (range 13 to 64%) of the total lymphocyte count.
Interestingly, 6 patients had received Rituximab (for lymphoma in 2, for pSS systemic manifestations or cryoglobulinemia in 4). The median time between LGL proliferation diagnosis and first Rituximab injection was 7 months (range 15 days to 13 months). Neutropenia occurred in 5/6 patients having received RTX. One patient with neutropenia, was retreated with Rituximab for a cryoglobulinemia relapse. Neutropenia occurred after the first injection (1200/mm3) and even worsened after the second (700/mm3).
In 2 patients, severe neutropenia required treatment by G-CSF which only led to transient efficacy between the injections. Methotreaxate was added in these 2 patients and allowed normalization in one case whereas neutrophil count partly improved in the other case. Spontaneous remission occurred in 1/7 patients and non-severe neutropenia persisted in 4/7 others at last evaluation.
This is one of the largest study analyzing LGL proliferation in pSS. As in other auto-immune diseases, LGL were mainly T-LGL, their main feature is neutropenia and they were observed in patients with high activity. Interestingly, the majority occurred in patients treated by Rituximab and could have a link with this treatment. This study suggest that neutropenia occurring after Rituximab treatment in patients with lymphoma or systemic auto-immune disease (but rarely in patients with rheumatoid arthritis) could be the consequence of a minimal LGL proliferation that should be searched in this context.
To cite this abstract in AMA style:Baber A, Nocturne G, Mariette X, Seror R. Large Granular Lymphocyte Proliferations in Primary Sjögren’s Syndrome: An Iatrogenic Manifestation [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/large-granular-lymphocyte-proliferations-in-primary-sjogrens-syndrome-an-iatrogenic-manifestation/. Accessed August 12, 2020.
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