Felty Syndrome

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Autoimmune diseases can result in additional symptoms and complications impacting various organ systems beyond the joints. These can affect the eyes, skin, respiratory, cardiac, and renal systems. Recognizing and understanding these diverse manifestations, such as the severe eye issues seen in rheumatoid arthritis (RA) and the potentially life-threatening Felty syndrome, is crucial for clinicians to promptly identify and treat these conditions effectively. In this case presentation, we report on a patient admitted for bilateral scleritis, which was found to be secondary to multiple autoimmune syndrome type 3...

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Neutrophilic dermatosis of the dorsal hands (NDDH) is a variant of Sweet syndrome that presents with erythematous bullae, papules/plaques, or pustules on the dorsal hands. It is most commonly associated with hematologic and solid organ malignancies, though cases of NDDH associated with inflammatory bowel disease, rheumatologic disorders, and medication exposure have also been described in the literature. Felty syndrome is a rare complication of long-standing rheumatoid arthritis characterized by neuropathy, splenomegaly, and neutropenia...

Felty's syndrome was first described in 1924 by the US-American physician Augustus Roi Felty as a triad of rheumatoid arthritis, splenomegaly and leucopenia. Even nearly 100 years later, this rare syndrome is still paralleled by diagnostic and therapeutic challenges and its pathogenesis is incompletely understood. Neutropenia with potentially life-threatening infections is the main problem and several pathomechanisms like Fas-mediated apoptosis, anti-neutrophil antibodies, anti-G-CSF antibodies, neutrophil consumption in the context of NETosis and suppression of granulopoiesis by T-LGLs have been suggested...

Felty syndrome is characterized by a triad of rheumatoid arthritis, neutropenia, and splenomegaly that typically occurs in patients with seropositive rheumatoid arthritis. We report a case of an 81-year-old man with a 22-year history of rheumatoid arthritis who developed pancytopenia and neutropenia for one month. An abdominal ultrasound revealed splenomegaly. A bone marrow biopsy excluded large granular lymphocytic leukemia, thus Felty Syndrome was diagnosed. He was treated with a maximum tolerated dose of methotrexate (15 mg once a week) for two months with no improvement in absolute neutrophil count...

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Large granular lymphocytic (LGL) leukemia is a chronic lymphoproliferative disease of cytotoxic T cells or NK cells with LGL morphology and frequently complicated cytopenia and/or different autoimmune diseases, which often require medical interventions, although LGL leukemia itself is seldom lethal. Immunologic dysregulations in LGL leukemia contribute to the development of complications, for example, neutropenia with the involvement of Fas ligand system and, in pure red cell aplasia, which is a common complication among the patients of East Asian origin, impairing erythroid developments by cytotoxic T cells...

T-cell large granular lymphocytic (LGL) leukemia is characterized by a clonal proliferation of CD3+ T-cells and has been associated with rheumatoid arthritis (RA). Splenomegaly is a common finding and a majority of cases present with cytopenia. Felty syndrome (FS) is characterized by neutropenia and splenomegaly and is also classically described in the literature for its association with RA. Similarities in clinical features, pathogenesis, management, genetics, and immunologic basis of FS and T-cell LGL leukemia have led to the suggestion that they exist on the same spectrum of disease...

OBJECTIVE: This work aims to analyze the quantity and expenses related to biological drugs used for the treatment of rheumatoid arthritis (RA) in outpatient public care within the Brazilian Unified Health System (SUS). METHODS: It is a cross-sectional descriptive study based on secondary data from a historical series, referring to the purchase, volume, and the number of patients treated with different biological drugs (infliximabe, etanercept, adalimumab, rituximab, abatacept, tocilizumab, golimumab, and certolizumab pegol) for RA treatment in outpatient care from 2012 to 2017...

A triad of symptoms characterises Felty's syndrome: seropositive rheumatoid arthritis (RA), splenomegaly and neutropenia. The treatment of Felty's syndrome is based on using classic synthetic and biological disease-modifying anti-rheumatic drugs (DMARDs). In this article, we present a case of a patient with Felty's syndrome who was treated with biologic treatment. A systematic search of the literature on the electronic medical database was conducted. The drugs from the DMARD group, despite reducing the activity of the disease, may cause significant clinical complications...

Mycobacterium xenopi is a rare cause of spinal osteomyelitis and discitis. Here we report the case of a 68-year-old woman with history of splenectomy for Felty's syndrome who developed M. xenopi lumbar discitis and osteomyelitis following repeated corticosteroid spinal injections for chronic back pain. Review of the 18 cases of M. xenopi spinal osteomyelitis cases described in the literature revealed common threads of immunocompromised hosts and prior spinal manipulation.

OBJECTIVES: Neutropenia is a key presentation of Felty syndrome (FS) and rheumatoid arthritis (RA)-associated T-cell large granular lymphocytic (T-LGL) leukaemia. Clonal rearrangement of T-cell receptor (TCR) gene supports the diagnosis of T-LGL leukaemia but not FS. Mutations in the signal transducer and activator of transcription 3 (STAT3) gene are highly specific for T-LGL leukaemia, but their prevalence in FS remains poorly clarified. METHODS: The study included 100 patients with RA and unexplained neutropenia...

Advanced rheumatoid arthritis (RA) is complicated by extra-articular manifestations such as small- and medium-sized vasculitis, pulmonary fibrosis, and pleurisy. The clinical course of the disease is refractory and critical. Treating advanced RA with multiple extra-articular manifestations is challenging. Here, we report a case of advanced RA in a 75-year-old man with exudative pleurisy and Felty syndrome. Treatment should be initiated promptly while paying attention to the possibility of infection as a differential diagnosis of exudative pleurisy because of the drastic change in the patient's condition due to disease progression...

Hairy cell leukemia (HCL) is a rare malignancy that primarily affects the bone marrow, peripheral blood, and spleen. The most common presenting features of HCL are splenomegaly or cytopenias causing fatigue, infections, or hemorrhagic manifestations. Symptoms involving the soft tissue or bone are rare. HCL can rarely present with immune-mediated polyarthritis. This presentation can be confused for other pathological entities, such as Felty's syndrome, and can be differentiated from this with bone marrow biopsy...

BACKGROUND: Felty syndrome is defined by three conditions: neutropenia, rheumatoid arthritis, and splenomegaly. Neutropenia associated with pancytopenia may further affect the dental condition of a patient. Periodontal treatment and surgery in patients with Felty syndrome necessitates cooperation with a hematologist. Here we present a case of a patient with Felty syndrome who was initially referred to the oral surgery hospital attached to the School of Dentistry for extensive periodontitis...

T cell large granular lymphocyte leukemia (T-LGLL) is an interesting case at the intersection of autoimmunity and cancer. In T-LGLL, T cells with somatic pathogenic mutations (mainly in STAT3) are linked to rheumatoid arthritis (RA) and neutropenia. A rare subtype of RA, Felty's syndrome, exhibits overlapping clinical features and comparable frequencies of activating STAT3 mutations in T cells as T-LGLL, which hints at a potential T-LGLL-Felty's syndrome-RA axis. Somatic mutations could shed light on the unexplained pathologies of these disorders...

Felty syndrome (FS) is a disorder that develops after a long history of erosive rheumatoid arthritis and presents with splenomegaly and neutropenia. In addition to joint deformities, FS causes a variety of extra-articular manifestations such as vasculitis, skin lesions, and lymphadenopathy. FS is also reported to cause non-cirrhotic portal hypertension which may result in variceal bleeding. FS is usually treated by disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate. Herein, we report a case of rapidly deteriorating FS and a severe relapsing neutropenia only a few weeks after discontinuation of methotrexate and other available DMARDs...

A complex relationship exists between rheumatic diseases and cancer. This delicate balance between chronic inflammation and malignant cell transformation in hematologic neoplasms has been observed, but is not well defined. Large Granular Lymphocyte (LGL) leukemia is at the intersection of a clonal lymphoproliferative disease, chronic inflammation, and autoimmunity. The association between rheumatoid arthritis (RA) and the spectrum of Felty's Syndrome is well-known. Other rheumatic disorders have been reported including systemic lupus erythematosus (SLE), Sjogren's Syndrome (SS), vasculitis, Behcet's Disease (BD) and systemic sclerosis...

The authors report the case of a 55-year-old patient with a chronic lower-limb wound thought to be secondary to vasculitis. This case illustrates the importance of maintaining a high index of suspicion for vasculitic ulcers in patients with autoimmune disease. Management considerations in this context are also discussed.