Intersection Between Large Granular Lymphocyte Leukemia and Rheumatoid Arthritis

Moosic, Katharine B.; Ananth, Kusuma; Andrade, Felipe; Feith, David J.; Darrah, Erika

doi:10.3389/fonc.2022.869205

Cited by 11 publications

(8 citation statements)

References 105 publications

(133 reference statements)

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“…Despite the high prevalence of autoimmune disorders in T-LGL leukemia patients, our patient did not present any symptoms or serological anomalies indicating the presence of autoimmune disease [5,6].…”

Section: A Diagnosis Of T-contrasting

confidence: 58%

Febrile Neutropenia as the First Manifestation of T-Cell Large Granular Lymphocytic Leukemia

Martins¹,

Silva²,

Gomes³

et al. 2022

Cureus

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T-cell large granular lymphocytic (T-LGL) leukemia is a rare lymphoproliferative disorder, characterized by peripheral blood and bone marrow infiltration with large granular lymphocytes (LGL), splenomegaly, cytopenias, and a frequent association with autoimmune diseases. Recurrent bacterial infections due to neutropenia are the main reason why patients come to medical attention. Despite not being a curable disease, T-LGL leukemia usually has an indolent course, with deaths mainly resulting from severe infections. Treatment is often not required, however, when needed, aims to relieve symptoms, and reduce infections and transfusion needs.We describe a case of an 86-year-old female patient with febrile neutropenia, diagnosed with T-LGL leukemia after the resolution of infection and exclusion of other causes of neutropenia. A "watch and wait" approach was established after a multidisciplinary discussion.This case shows a frequent presentation of a rare disease, as well as the approach from diagnosis to treatment, reminding clinicians that T-LGL leukemia should be considered in the differential diagnosis of adults with febrile neutropenia.

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Section: A Diagnosis Of T-contrasting

confidence: 58%

Febrile Neutropenia as the First Manifestation of T-Cell Large Granular Lymphocytic Leukemia

Martins¹,

Silva²,

Gomes³

et al. 2022

Cureus

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“…TLGL leukemia is an extremely rare lymphoproliferative disorder that can occur in association with autoimmune and hematologic disorders, most commonly RA [1]. The pathophysiology behind TLGL leukemia is poorly understood but has been noted to be associated with dysregulated signaling related to the mass proliferation of immune cells and dysregulated apoptotic pathways associated with chronic inflammation [4].…”

Section: Discussionmentioning

confidence: 99%

“…T-cell large granulocyte lymphocytic (TLGL) leukemia is a lymphoproliferative disorder that can occur in association with rheumatoid arthritis (RA) [1]. The disease course is typically indolent and thus can develop over a long time course prior to symptoms becoming apparent [2].…”

Section: Introductionmentioning

confidence: 99%

A Case of T-Cell Large Granulocyte Lymphocytic Leukemia in Rheumatoid Arthritis

Robinson¹,

Ramanan²,

Singh³

et al. 2023

Cureus

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T-cell large granulocyte lymphocytic (TLGL) leukemia is a lymphoproliferative disorder involving clonal expansion of cytotoxic T-cells and subsequent cytopenia, most notably neutropenia, as well as splenomegaly. TLGL leukemia is commonly associated with autoimmune disorders, most commonly rheumatoid arthritis (RA). We present a case of a 54-year-old female with a past medical history of seropositive RA who was lost to follow-up and thus was not on any active treatment for RA for several years. She returned to the clinic with worsening joint pain, swelling, and stiffness involving multiple joints. Screen laboratory work revealed an absolute neutrophil count (ANC) of 0.19 K/uL, indicating severe neutropenia. This finding prompted further workup, for which our patient was ultimately diagnosed with TLGL leukemia. Proper treatment and control of inflammation in RA are important not only to preserve joint function and vitality but also to prevent rare sequela of untreated autoimmune disorders, as was the case in our patient.

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“…A minority of RA patients may also develop Felty’s syndrome, a disorder characterized by the typical triad of RA, neutropenia, and splenomegaly. Remarkably, up to 40% of FS cases present a concomitant LGLL [ 57 , 58 ] and, in this specific instance, the integration of clinical manifestations with signs of the autoimmune disease might help in defining the nature of cytopenias [ 59 ].…”

Section: The Boundaries Between Lgll and Autoimmunity In The Pathogen...mentioning

confidence: 99%

LGL Clonal Expansion and Unexplained Cytopenia: Two Clues Don’t Make an Evidence

Calabretto

Attardi

Gurnari

et al. 2022

Cancers

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Clonal expansions of large granular lymphocytes (LGL) have been reported in a wide spectrum of conditions, with LGL leukemia (LGLL) being the most extreme. However, the boundaries between LGLL and LGL clones are often subtle, and both conditions can be detected in several clinical scenarios, particularly in patients with cytopenias. The intricate overlap of LGL clonal expansion with other disease entities characterized by unexplained cytopenias makes their classification challenging. Indeed, precisely assigning whether cytopenias might be related to inadequate hematopoiesis (i.e., LGL as a marginal finding) rather than immune-mediated mechanisms (i.e., LGLL) is far from being an easy task. As LGL clones acquire different pathogenetic roles and relevance according to their diverse clinical settings, their detection in the landscape of bone marrow failures and myeloid neoplasms has recently raised growing clinical interest. In this regard, the current availability of different diagnostic techniques, including next generation sequencing, shed light on the relationship between LGL clones and cytopenias, paving the way towards a better disease classification for precision medicine treatments. Herein, we discuss the clinical relevance of LGL clones in the diagnostic algorithm to be followed in patients presenting with cytopenias, offering a foundation for rational management approaches.

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Intersection Between Large Granular Lymphocyte Leukemia and Rheumatoid Arthritis

Cited by 11 publications

References 105 publications

Febrile Neutropenia as the First Manifestation of T-Cell Large Granular Lymphocytic Leukemia

Febrile Neutropenia as the First Manifestation of T-Cell Large Granular Lymphocytic Leukemia

A Case of T-Cell Large Granulocyte Lymphocytic Leukemia in Rheumatoid Arthritis

LGL Clonal Expansion and Unexplained Cytopenia: Two Clues Don’t Make an Evidence

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