Large granular lymphocyte expansions in patients with Felty's syndrome: analysis using anti-T cell receptor Vβ-specific monoclonal antibodies

“…In fact, approximately 30–40% of FS patients have peripheral blood expansions of large granular lymphocytes [7]. The clinical presentation in FS patients closely resembles LGL leukemia patients with neutropenia and RA [6].…”

Section: Introductionmentioning

confidence: 99%

The spectrum of large granular lymphocyte leukemia and Feltyʼs syndrome

Liu

Loughran

2011

Current Opinion in Hematology

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Purpose of review Patients with chronic large granular lymphocyte (LGL) leukemia often have rheumatoid arthritis, neutropenia and splenomegaly, thereby resembling the manifestations observed in patients with Felty’s syndrome (FS), which is a rare complication of rheumatoid arthritis (RA) characterized by neutropenia and splenomegaly. Both entities have similar clinical and laboratory presentation, as well as common genetic determinant HLA-DR4, indicating they may be part of the same disease spectrum. This review paper seeks to discuss the underlying pathogenesis and therapeutic algorithm of RA, neutropenia and splenomegaly in the spectrum of LGL leukemia and Felty’s syndrome. Recent findings We hypothesize that there may be a common pathogenic mechanism between LGL leukemia and typical FS. Phenotypic and functional data have strongly suggested that CD3+ LGL leukemia are antigen-activated. Aberrations in the T cell repertoire with the emergence of oligoclonal/clonal lymphoid populations have been found to play a pivotal role in pathogenesis of rheumatoid arthritis. The biologic properties of the pivotal T cell involved in rheumatoid arthritis pathogenesis are remarkably similar to those in leukemic LGL. Summary RA-associated T-LGL leukemia and articular manifestations of typical Felty’s syndrome are not distinguishable. A common pathogenetic link between LGL leukemia and RA is proposed.

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“…LGL leukaemia must be considered, since 30%–40% of FS cases may have LGL expansions 7. However, this was not reported in any of the cases we reviewed.…”

Section: Discussionmentioning

confidence: 85%

Neutropaenia and splenomegaly without arthritis: think rheumatoid arthritis

Aslam¹,

Cheema

Feinstein

et al. 2018

BMJ Case Reports

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Felty syndrome(FS) is an uncommon, but severe, extra-articular manifestation of rheumatoid arthritis (RA). It occurs in patients with longstanding RA. It is extremely rare for RA to present as FS or develop after initially presenting as neutropaenia and splenomegaly. We describe a case of 47-year-old woman who was diagnosed simultaneously with FS and possible RA after testing positive for anticyclic citrullinated peptide antibody, but a negative rheumatoid factor. She had an excellent response to methotrexate. We review the existing literature of such cases and emphasise the importance of serological testing for RA in patients presenting with neutropaenia and splenomegaly, even in the absence of joint symptoms or prior diagnosis of RA.

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Large granular lymphocyte expansions in patients with Felty's syndrome: analysis using anti-T cell receptor Vβ-specific monoclonal antibodies

Cited by 44 publications

References 36 publications

A comparative study of flow cytometric T cell receptor Vβ repertoire and T cell receptor gene rearrangement in the diagnosis of large granular lymphocytic lymphoproliferation

A comparative study of flow cytometric T cell receptor Vβ repertoire and T cell receptor gene rearrangement in the diagnosis of large granular lymphocytic lymphoproliferation

The spectrum of large granular lymphocyte leukemia and Feltyʼs syndrome

Neutropaenia and splenomegaly without arthritis: think rheumatoid arthritis

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