scholarly journals T-CELL LARGE GRANULAR LYMPHOCYTE LEUKEMIA, A RARE MANIFESTATION OF RHEUMATOID ARTHRITIS: CASE REPORT

2019 ◽  
Author(s):  
PRISCILA GARCIA CÂMARA CABRAL TAVARES ◽  
ANA PAULA DE MENEZES CUNHA ◽  
RUY SAMPAIO SIQUEIRA NETO ◽  
ANDRÉ DE GOIS ROCHA ◽  
LEILA PATRÍCIA FONSECA OLIVEIRA ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Case Report ◽  
T Cell ◽  
Large Granular Lymphocyte ◽  
Rheumatoid Arthritis Case ◽  
Large Granular Lymphocyte Leukemia ◽  
Rare Manifestation

scholarly journals The case of T-cell large granular lyphocyte leukemia presented as transfusion dependent anemia with sustained response to cyclosporine a therapy: Case report

2016 ◽  
Vol 69 (11-12) ◽  
pp. 376-378 ◽  
Author(s):  
Vanja Zeremski ◽  
Aleksandar Savic ◽  
Vesna Cemerikic-Martinovic ◽  
Ivana Milosevic ◽  
Marina Dragicevic ◽  
...  
Keyword(s):  
Case Report ◽  
T Cell ◽  
Cell Receptor ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocytes ◽  
Large Granular Lymphocyte Leukemia ◽  
T Cell Receptor Genes ◽  
Cyclosporine Therapy ◽  
Dose Oral ◽  
Granular Lymphocytes

Case report. A 41-year-old man presented with anemia, lymphocytosis and splenomegaly. T-cell large granular lymphocyte leukemia was diagnosed based on lymphocytosis of T-cell large granular lymphocytes, characteristic immunophenotype (CD3+, CD8+, CD16+, CD57+) of the lymphocytes and clonally rearranged T-cell receptor genes. Therapy indication was transfusion-dependent anemia. Initial cyclosporine therapy and low-dose oral methotrexate failed to control anemia and lymphocytosis. Yet, a complete clinical and hematological response (without molecular remission) was achieved and sustained when cyclosporine was reintroduced into the therapy. Conclusion. Our case confirms that cyclosporine therapy is effective in treating T-cell large granular lymphocyte leukemia and suggests that indefinite treatment may not be needed to maintain the response.

Impact of MICA and Other Immunogenetic Polymorphisms On Clinical Presentation of T Cell Large Granular Lymphocyte Leukemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1094-1094
Author(s):  
Aleksandr Lazaryan ◽  
Nelli Bejanyan ◽  
Aaron D. Viny ◽  
Medhat Askar ◽  
Pearlie K Burnette ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Overall Survival ◽  
T Cell ◽  
Cell Count ◽  
Clinical Outcomes ◽  
Clinical Presentation ◽  
Response To Therapy ◽  
Large Granular Lymphocyte ◽  
Class I ◽  
Large Granular Lymphocyte Leukemia

Abstract Abstract 1094 Poster Board I-116 T cell large granular lymphocyte leukemia (T-LGL) is a rare lymphoproliferative disorder marked by clonal expansion of cytotoxic T lymphocytes (CTL). T-LGL may be a result of clonal outgrowth from initially polyclonal CTL responses seen in the context of viral infections, autoimmune conditions, or tumor surveillance. Similar to classic autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis or aplastic anemia, immunogenetic predisposition to T-LGL is suggested by association with certain immunogenetic polymorphisms including human leukocyte antigen (HLA) and killer immunoglobulin receptor (KIR). In addition to KIR-ligand/KIR interactions, the quality of CTL may be determined by the binding between the major histocompatibility complex class I chain-related gene A (MICA) and its ligand NKG2A. Over fifty five MICA alleles have been documented to date. A number of autoimmune and oncologic conditions such as systemic lupus erythematosus, rheumatoid arthritis, psoriasis, Crohn's disease, cervical cancer, or oral squamous cell carcinoma were all reported to be associated with MICA polymorphism. We hypothesized that specific MICA polymorphisms may be associated with exaggerated CTL responses in T-LGL and may therefore impact clinical features of the disease such as immune cytopenias. We have collected a large well annotated cohort of patients with T-LGL (n=86). HLA, MICA, and KIR alleles were resolved by established molecular techniques. Diagnosis of T-LGL was established by flow cytometry, T cell receptor γ chain rearrangement, Vβ typing, and assessment of peripheral blood smear. Categorical and survival methods of data analysis were used to examine the association between MICA, HLA, and KIR polymorphisms with type and degree of cytopenias, LGL T cell count, response to therapy, splenomegaly, and overall survival. Caucasians accounted for 96.5% of the study cohort (median age, 64 years; 55% males). Neutropenia, anemia, and thrombocytopenia were found in 63.4%, 50%, and 23.5% of the patients, respectively. Bicytopenia and pancytopenia were found in 26% and 13% of subjects, respectively. Median LGL T cell count was 1800 cells/μL (range, 280-20,580 cells/μL). Splenomegaly was found in 47% of patients. Compared to healthy controls (2N=308), our cohort was overrepresented by MICA*A5.1 (population frequency, 0.59 in T-LGL vs. 0.37 in controls, p<0.001). As opposed to the patients carrying MICA*A4 (p=0.01), those with MICA*A5.1 (p=0.025) or MICA*008 (p=0.025) were less likely to present with splenomegaly. MICA*A6 carriers were more likely to have thrombocytopenia (p=0.04). Since immunogenetic predisposition is often encoded by complex genetic traits, we have also examined the impacts of HLA class I, II and KIR-ligand/ KIR on clinical presentation of T-LGL. Immunogenetic factors associated with anemia as a predominant feature included HLA-DR7 (p=0.003), Bw4-KIR3DL1 (p=0.03), or having less than 3 inhibitory KIR ligand mismatches (p=0.025). Subjects with HLA-Bw6 (p=0.02) and HLA-A*24 (p=0.05) were more likely to have neutropenia, whereas the patients with HLA*A11 were more likely to have thrombocytopenia (p=0.01). HLA-A*24 (p=0.004), HLA-B*15 (p=0.03), and KIR2DL5 (p=0.045) were associated with poorer clinical response to therapy in contrast to those with HLA*01 (p=0.008) and HLA-B*58 (p=0.045) associated with more favorable clinical responses. In contrast to HLA-B*27 carriers with higher likelihood of splenomegaly (p=0.01), patients with HLA-Bw4-KIR3DL1 (p=0.046), HLA-DR3 (p=0.005), or HLA-DQ2 (p=0.028) were less likely to develop splenomegaly. We did not detect any associations with overall survival. In summary, the overrepresentation of MICA*A5.1 among T-LGL patient had limited impact on clinical outcomes. In contrast to numerous associations between HLA and KIR with clinical presentation of T-LGL, the associations for MICA alleles in our cohort were limited to less likelihood of splenomegaly for MICA*A5.1 and MICA*008 carriers and to higher propensity towards thrombocytopenia for those with MICA*A6. Altogether, our findings extend the evidence for a role of immunogenetic factors in the pathogenesis and clinical outcomes of T-LGL. Disclosures No relevant conflicts of interest to declare.

scholarly journals CD20-Positive T-Cell Large Granular Lymphocyte Leukemia: Case Report and Review of the Literature

2009 ◽  
Vol 48 (16) ◽  
pp. 1443-1447 ◽  
Author(s):  
Koji Miyazaki ◽  
Manabu Ohsaka ◽  
Yuhko Suzuki ◽  
Mikio Danbara ◽  
Ryouichi Horie ◽  
...  
Keyword(s):  
Case Report ◽  
T Cell ◽  
Large Granular Lymphocyte ◽  
Review Of The Literature ◽  
Large Granular Lymphocyte Leukemia

Biclonal T-cell receptorgammadelta+ large granular lymphocyte leukemia associated with rheumatoid arthritis

2008 ◽  
Vol 49 (4) ◽  
pp. 828-831 ◽  
Author(s):  
Monika Prochorec-Sobieszek ◽  
Monika Chełstowska ◽  
Grzegorz Rymkiewicz ◽  
Mirosław Majewski ◽  
Krzysztof Warzocha ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
T Cell ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocyte Leukemia

scholarly journals T-Cell Large Granular Lymphocyte Leukemia in a Patient With Rheumatoid Arthritis

2020 ◽  
Vol 8 ◽  
pp. 232470962094130
Author(s):  
Sara Naji Rad ◽  
Behnam Rafiee ◽  
Gagan Raju ◽  
Mahdis Solhjoo ◽  
Prachi Anand
Keyword(s):  
Rheumatoid Arthritis ◽  
T Cell ◽  
Clinical Manifestations ◽  
Cell Lineage ◽  
Common Type ◽  
Large Granular Lymphocyte ◽  
Disease Course ◽  
Large Granular Lymphocyte Leukemia ◽  
Disease Spectrum ◽  
Indolent Disease

Large granular lymphocyte leukemia (LGL) is a clonal, lymphoproliferative disorder with an indolent disease course. T-cell LGL (T-LGL) is the most common type of LGL driven from T-cell lineage (85%). The coexistence of T-LGL with several types of autoimmune disorders, mostly rheumatoid arthritis (RA), has been reported. Felty’s syndrome (FS) is defined by splenomegaly, low neutrophil count, and destructive arthritis and is usually seen in <1% of patients with RA. About 30% to 40% of patients with FS have been reported to have an expansion of large granulated lymphocytes in the circulation. FS and T-LGL are similar in terms of clinical manifestations, response to immunosuppressive therapy, their smoldering course, and immunogenetic findings, proposing FS and T-LGL with RA might be different aspects of a single disease spectrum. In this article, we present a case with long-standing RA who had never been on DMARD (Disease Modifying Anti-Rheumatic Drugs) treatment found to have constitutional symptoms, neutropenia, and splenomegaly, and the patient was diagnosed with T-LGL.

Long-term remission of T-cell large granular lymphocyte leukemia associated with rheumatoid arthritis after rituximab therapy

Blood ◽  
2013 ◽  
Vol 122 (9) ◽  
pp. 1583-1586 ◽  
Author(s):  
Divi Cornec ◽  
Valérie Devauchelle-Pensec ◽  
Sandrine Jousse-Joulin ◽  
Thierry Marhadour ◽  
Valérie Ugo ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
T Cell ◽  
Effective Therapy ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocyte Leukemia ◽  
Key Points

Key Points Rituximab may be a safe and effective therapy in patients with both large granular lymphocyte leukemia and rheumatoid arthritis. LGLL in some patients with rheumatoid arthritis may be a reactive manifestation of chronic autoantigen stimulation rather than a true concomitant malignancy.

Rheumatoid arthritis and T cell large granular lymphocyte leukaemia: A case report

2016 ◽  
Vol 12 (4) ◽  
pp. 239-240
Author(s):  
María del Mar Herráez-Albendea ◽  
María del Castillo Jarilla-Fernández ◽  
Francisco Jiménez-Burgos ◽  
Elisa Sánchez-Rodriguez
Keyword(s):  
Rheumatoid Arthritis ◽  
Case Report ◽  
T Cell ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocyte Leukaemia

scholarly journals T-CELL LARGE GRANULAR LYMPHOCYTE LEUKEMIA: A CASE REPORT

2019 ◽  
Author(s):  
SANDRA HELENA MACHADO ◽  
YASMINNE ROCHA ◽  
FERNANDA BECKER TABAJARA ◽  
JOÃO VICTOR DE ANDRADE AGUAS ◽  
LETICIA ROSSETTO DAUDT ◽  
...  
Keyword(s):  
Case Report ◽  
T Cell ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocyte Leukemia

Large Granular Lymphocyte Leukemia: Case Report of Chronic Neutropenia and Rheumatoid Arthritis-like Symptoms in a Child

2001 ◽  
Vol 4 (1) ◽  
pp. 94-99 ◽  
Author(s):  
Carol A. Blanchong ◽  
Randal Olshefski ◽  
Samir Kahwash
Keyword(s):  
Rheumatoid Arthritis ◽  
T Cell ◽  
Receptor Gene ◽  
Lymphoproliferative Disorders ◽  
Large Granular Lymphocyte ◽  
Large Granular Lymphocytes ◽  
Large Granular Lymphocyte Leukemia ◽  
Granular Lymphocytes ◽  
Chronic Neutropenia ◽  
Lgl Leukemia

Lymphoproliferative disorders of large granular lymphocytes (LGL) are heterogeneous, with a clinical/pathologic spectrum ranging from a benign polyclonal expansion to an aggressive clonal disease. Often these lymphoproliferative disorders are associated with autoimmune disease. The clonal form of the disorder, LGL leukemia, typically occurs in older adults with a median age of 55 years at diagnosis. Pediatric cases are referred to in review articles; however, no detailed reports of T-cell LGL leukemia in children exist. This report illustrates a case of a child who presented initially at age 2 and 1/2 years with psoriasis, juvenile rheumatoid arthritis-like symptoms, and neutropenia. Bone marrow examinations obtained throughout his course have demonstrated progressive hypercellularity with increased reticulin fibers and replacement of the normal marrow elements by lymphocytes, which were later identified as large granular lymphocytes. Further testing with immunophenotyping by flow cytometry and T-cell receptor gene rearrangement studies revealed a monoclonal proliferation of large granular lymphocytes and confirmed a diagnosis of LGL leukemia. Although rare, large granular lymphocyte leukemia should be included in the differential diagnosis of chronic neutropenia in children.

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