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Felty Syndrome

Roberto Cupaiolo, Soraya Cherifi, Christophe Lelubre, Danielle Govaerts, Quentin Delefortrie, Francis Corazza, Charles Chevalier
Large granular lymphocyte leukemia (LGL) are chronic lymphoproliferative disorders classified into three main groups: T-cell LGL leukemia (T-LGL), aggressive NK-cell leukemia and chronic lymphoproliferative disorder of NK cells (NK-LGL). Patients with LGL leukemia exhibit chronic (>3 months) and moderate (<1G/L) to substantial monoclonal expansion of large granular lymphocytes in the peripheral blood. Cytologically, large granular lymphocytes are medium to large cells which are further characterized by an eccentric nucleus and a slightly basophilic cytoplasm containing azurophilic granules...
August 1, 2018: Annales de Biologie Clinique
Fawad Aslam, Rabia S Cheema, Michael Feinstein, April Chang-Miller
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...
July 11, 2018: BMJ Case Reports
José Kennedy Amaral, Robert T Schoen
INTRODUCTION: Chronic chikungunya (CHIK) arthritis, an inflammatory arthritis, often follows acute CHIK fever (CHIKF), a viral infection. The pathogenesis of chronic CHIK arthritis is poorly characterized, but may resemble other forms of inflammatory arthritis. Clinically, chronic CHIK arthritis sometimes mimics rheumatoid arthritis (RA). CASE REPORT: We report a patient with well-characterized CHIKF followed 2 months later by chronic CHIK arthritis not only resembling RA clinically, but also associated with RA biomarkers and extra-articular features, including Felty's syndrome (FS)...
June 2018: Rheumatology and Therapy
George D Liatsos, Ioanna Tsironi, Dimitrios Vassilopoulos, Spyridon Dourakis
In severe cases of pancytopenia with subsequent infections due to long-term untreated Felty's syndrome, the initiation of immunosuppressive treatment with sole prednisone (1 mg/kg iv ) should be considered, despite that, the low neutrocytes count would make one physician hesitant. A full resolution of whole blood count within 3 weeks and a 30% reduction in spleens sized was noted.
March 2018: Clinical Case Reports
Viviana Lora, Lorenzo Cerroni, Carlo Cota
Rheumatoid arthritis is a chronic systemic disease that, in addition to articular involvement, may exhibit a variety of extraarticular manifestations. The skin is frequently involved mainly in the most severe forms of the disease. Rheumatoid nodules, accelerated rheumatoid nodulosis, rheumatoid nodulosis, Felty syndrome, rheumatoid vasculitis, pyoderma gangrenosum, rheumatoid neutrophilic dermatosis, interstitial granulomatous dermatitis, and palisaded neutrophilic granulomatous dermatitis are reviewed both clinically and histologically...
April 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Tal Gazitt, Thomas P Loughran
This section reviews the diagnostic criteria and pathogenesis of large granular lymphocyte (LGL) leukemia. There is a particular focus on the overlap of LGL leukemia and rheumatoid arthritis (Felty's syndrome). Current understanding of the mechanisms of neutropenia in these disorders is discussed. Finally, treatment indications and therapeutic recommendations are outlined.
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Paula Savola, Oscar Brück, Thomas Olson, Tiina Kelkka, Markku J Kauppi, Panu E Kovanen, Soili Kytölä, Tuulikki Sokka-Isler, Thomas P Loughran, Marjatta Leirisalo-Repo, Satu Mustjoki
Felty syndrome is a rare disease defined by neutropenia, splenomegaly, and rheumatoid arthritis. Sometimes the differential diagnosis between Felty syndrome and large granular lymphocyte leukemia is problematic. Recently, somatic STAT3 and STAT5B mutations were discovered in 30-40% of patients with large granular lymphocyte leukemia. Herein, we aimed to study whether these mutations can also be detected in Felty syndrome, which would imply the existence of a common pathogenic mechanism between these two disease entities...
February 2018: Haematologica
C Kneitz, J Atta, H Burkhardt
Hematological alterations can often be observed during rheumatic diseases. The effects can be clinically severe, ranging from anemia of different grades of severity, through increased risk of hemorrhage due to thrombocytopenia up to severe infections as a result of high-grade leukocytopenia. The clinical sequelae for patients are predominantly determined by the extent of cytopenia. The underlying disease itself can initially be considered as the cause. Examples are anemia as a result of chronic inflammation, antibody-mediated thrombocytopenia as in systemic lupus erythematosus (SLE) or granulocytopenia within the framework of Felty's syndrome...
October 2017: Zeitschrift Für Rheumatologie
C-R Wang, Y-C Chiu, Y-C Chen
No abstract text is available yet for this article.
July 2018: Scandinavian Journal of Rheumatology
Nishant Dwivedi, Annica Hedberg, Ying Yi Zheng, Indira Neeli, Minoru Satoh, Laurence Morel, Ole Petter Rekvig, Marko Radic
Deimination, a posttranslational modification of arginine to citrulline carried out by peptidylarginine deiminases, may compromise tolerance of self-antigens. Patients with connective tissue autoimmunity, particularly rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or Felty's syndrome, present with autoantibodies to deiminated histones (dH), which thus form a category of antibodies to citrullinated protein antigens (ACPA). In general, ACPA are a sensitive diagnostic for RA and may form in response to the release of nuclear chromatin (DNA plus dH) from granulocytes, usually referred to as neutrophil extracellular traps...
2017: Frontiers in Immunology
Klaus Stahl, Michelle Duong, Anke Schwarz, A D Wagner, Hermann Haller, Mario Schiffer, Roland Jacobs
Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab...
2017: Case Reports in Nephrology
Nazim Benzerdjeb, Fatima Ameur, Jean-Fortune Ikoli, Henri Sevestre
Primary cardiac B cell lymphoma is rare. To date, fewer than 90 cases have been described in the literature. We report a 67-year-old woman with a 30-year history of rheumatoid arthritis, who had received treatment with leflunomide for 10 years and infliximab for 2 years. Secondary Felty's syndrome appeared. She was admitted to the hospital for abdominal pain. Investigations disclosed a 5cm cardiac mass in the right atrium. Histopathologic examination of tissue specimens obtained at surgical myocardial biopsy demonstrated primary cardiac B cell lymphoma...
December 2016: Pathology, Research and Practice
Preet Shah
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Ayten Yazıcı, Ayşenur Uçar, Özgür Mehtap, Emel Örge Gönüllü, Ali Tamer
An effective treatment strategy for Felty syndrome (FS) has not been developed so far. In this article, three cases with FS, who responded to different treatment modalities, have been presented. Case 1 was a 52-year-old male patient who initially received methotrexate, and then, he was switched to granulocyte colony-stimulating factor (G-CSF) and cyclosporine treatment when his neutropenia was further deteriorated. The patient needed monthly doses of G-CSF for nearly 6 months, and his steroid dose was increased...
September 2014: European Journal of Rheumatology
E C Sweeney
No abstract text is available yet for this article.
December 1975: Irish Journal of Medical Science
Oore-Ofe O Olumuyiwa-Akeredolu, Etheresia Pretorius
Adult rheumatoid arthritis (RA) is an autoimmune disorder affecting joints and frequently characterised by initial local and later systemic inflammation. Researchers have, for many years, traced its cause to diverse genetic, environmental and especially immunological responses that work against the body's own cells and tissues. Investigation into several of these biomarkers reveals interconnections that exist between multiple factors, which ultimately lead to specific pathologies. The goal of this paper is to highlight connections present between the major biological players long identified by researchers including more recently uncovered biomarkers in the RA repertoire and some of the pathophysiologies typically affiliated with the disease...
2016: Current Pharmaceutical Design
T Jain, C Mittal, R Sengupta, B Rubin
Felty's syndrome is a triad of rheumatoid arthritis, neutropenia, and splenomegaly. We hereby report an unusual case of non-articular Felty's syndrome and its management along with discussing the importance of appropriately ruling out alternate causes of neutropenia with splenomegaly.
November 2015: Netherlands Journal of Medicine
Mariana Lagrutta, Gelsomina Alle, Roberto Leandro Parodi, Alcides Alejandro Greca
Rheumatoid arthritis (RA) is a chronic autoimmune inflammatory disease occasionally associated with severe extra-articular manifestations, mostly in cases of longstanding highly active disease. We report the case of a 56 year-old woman diagnosed with active RA at the age of 40. After 5 years of high activity, her arthritis subsides spontaneously during pregnancy despite the lack of treatment with disease-modifying anti-rheumatic drugs. She remains without articular symptoms for 7 years, and then she develops a Felty's syndrome requiring steroid treatment and splenectomy...
July 2016: Reumatología Clinica
Estibaliz Lazaro, Jacques Morel
Neutropenia is defined as a neutrophil count lower than 1.5g/L, with categorization as mild, moderate, or severe when the count is 1.5-1g/L, 1-0.5g/L, or<0.5g/L, respectively. The main complication is infection, whose risk increases with the depth and duration of the neutropenia. Comprehensive etiological investigations are mandatory to determine the best treatment strategy. Constitutional neutropenia is rarely seen in everyday rheumatology practice. It predominantly affects patients of African descent and is usually moderate and well tolerated...
July 2015: Joint, Bone, Spine: Revue du Rhumatisme
En Shu, Hiroyuki Kanoh, Yusuke Kito, Mariko Seishima
No abstract text is available yet for this article.
July 2015: Acta Dermato-venereologica
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