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Acta Haematologica 2021Rheumatic diseases have many hematological manifestations. Blood dyscrasias and other hematological abnormalities are sometimes the first sign of rheumatic disease. In... (Review)
Review
BACKGROUND
Rheumatic diseases have many hematological manifestations. Blood dyscrasias and other hematological abnormalities are sometimes the first sign of rheumatic disease. In addition, novel antirheumatic biological agents may cause cytopenias.
SUMMARY
The aim of this review was to discuss cytopenias caused by systemic lupus erythematosus and antirheumatic drugs, Felty's syndrome in rheumatoid arthritis, and autoimmune hemolytic anemia, thrombosis, and thrombotic microangiopathies related to rheumatological conditions such as catastrophic antiphospholipid syndrome and scleroderma renal crisis. Key Message: The differential diagnosis of various hematological disorders should include rheumatic autoimmune diseases among other causes of blood cell and hemostasis abnormalities. It is crucial that hematologists be aware of these presentations so that they are diagnosed and treated in a timely manner.
Topics: Anemia, Hemolytic; Antirheumatic Agents; Felty Syndrome; Glucocorticoids; Granulocyte Colony-Stimulating Factor; Hematologic Diseases; Leukopenia; Lupus Erythematosus, Systemic; Protein Kinase Inhibitors; Rheumatic Diseases
PubMed: 33221805
DOI: 10.1159/000511759 -
Medical Principles and Practice :... 2018Rheumatoid arthritis (RA) is a chronic, inflammatory, systemic autoimmune disease, affecting the joints with varying severity among patients. The risk factors include... (Review)
Review
Rheumatoid arthritis (RA) is a chronic, inflammatory, systemic autoimmune disease, affecting the joints with varying severity among patients. The risk factors include age, gender, genetics, and environmental exposure (cigarette smoking, air pollutants, and occupational). Many complications can follow, such as permanent joint damage requiring arthroplasty, rheumatoid vasculitis, and Felty syndrome requiring splenectomy if it remains unaddressed. As there is no cure for RA, the treatment goals are to reduce the pain and stop/slow further damage. Here, we present a brief summary of various past and present treatment modalities to address the complications associated with RA.
Topics: Adrenal Cortex Hormones; Analgesics, Opioid; Anti-Inflammatory Agents, Non-Steroidal; Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Arthritis, Rheumatoid; Humans; Immunosuppressive Agents; Interleukin 1 Receptor Antagonist Protein; Leflunomide; Physical Therapy Modalities; Risk Factors; Tumor Necrosis Factor-alpha
PubMed: 30173215
DOI: 10.1159/000493390 -
Giornale Italiano Di Dermatologia E... Apr 2018Rheumatoid arthritis is a chronic systemic disease that, in addition to articular involvement, may exhibit a variety of extraarticular manifestations. The skin is... (Review)
Review
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. Moreover, cutaneous reactions related to treatments of rheumatoid arthritis, in particular to biologic agents, are described. Early recognition of these entities through a clinical and histological correlation permit to optimize the management of patients.
Topics: Arthritis, Rheumatoid; Biological Factors; Humans; Severity of Illness Index; Skin Diseases
PubMed: 29368864
DOI: 10.23736/S0392-0488.18.05872-8 -
Frontiers in Medicine 2023Felty'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... (Review)
Review
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's syndrome has various differential diagnoses as splenomegaly and cytopenia are common features of different infectious diseases, malignancies and autoimmune disorders. Additionally, benign clonal T-/NK-LGL lymphocytosis is increasingly noticed in Felty's syndrome, which further complicates diagnosis. Today's treatment options are still sparse and are largely based on case reports and small case series. Methotrexate is the mainstay of therapy, followed by rituximab, but there is less evidence for alternatives in the case of adverse reactions or failure of these drugs. This article gives an updated review about Felty's syndrome including its pathogenesis and treatment options.
PubMed: 37920595
DOI: 10.3389/fmed.2023.1238405 -
Current Opinion in Hematology Jul 2011Patients with chronic large granular lymphocyte (LGL) leukemia often have rheumatoid arthritis (RA), neutropenia and splenomegaly, thereby resembling the manifestations... (Review)
Review
PURPOSE OF REVIEW
Patients with chronic large granular lymphocyte (LGL) leukemia often have rheumatoid arthritis (RA), neutropenia and splenomegaly, thereby resembling the manifestations observed in patients with Felty's syndrome, which is a rare complication of RA characterized by neutropenia and splenomegaly. Both entities have similar clinical and laboratory presentation, as well as a 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 Felty's syndrome. Phenotypic and functional data have strongly suggested that CD3 LGL leukemia is 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 RA. The biologic properties of the pivotal T cell involved in RA pathogenesis are remarkably similar to those in leukemic LGL.
SUMMARY
RA-associated T-cell LGL leukemia and articular manifestations of typical Felty's syndrome are not distinguishable. A common pathogenetic link between LGL leukemia and RA is proposed.
Topics: Arthritis, Rheumatoid; Felty Syndrome; HLA-DR4 Antigen; Histocompatibility Testing; Humans; Leukemia, Large Granular Lymphocytic; Neutropenia
PubMed: 21546829
DOI: 10.1097/MOH.0b013e32834760fb -
The Open Rheumatology Journal 2014Felty's syndrome (FS) is characterized by the triad of seropositive rheumatoid arthritis (RA) with destructive joint involvement, splenomegaly and neutropenia. Current...
Felty's syndrome (FS) is characterized by the triad of seropositive rheumatoid arthritis (RA) with destructive joint involvement, splenomegaly and neutropenia. Current data shows that 1-3 % of RA patients are complicated with FS with an estimated prevalence of 10 per 100,000 populations. The complete triad is not an absolute requirement, but persistent neutropenia with an absolute neutrophil count (ANC) generally less than 1500/mm3 is necessary for establishing the diagnosis. Felty's syndrome may be asymptomatic but serious local or systemic infections may be the first clue to the diagnosis. FS is easily overlooked by parallel diagnoses of Sjӧgren syndrome or systemic lupus erythematosus or lymphohematopoietic malignancies. The role of genetic (HLA DR4) is more prominent in FS in comparison to classic rheumatoid arthritis. There is large body of evidence that in FS patients, both cellular and humoral immune systems participate in neutrophil activation, and apoptosis and its adherence to endothelial cells in the spleen. It has been demonstrated that proinflammatory cytokines may have inhibitory effects on bone marrow granulopoiesis. Binding of IgGs to neutrophil extracellular chromatin traps (NET) leading to neutrophil death plays a crucial role in its pathophysiology. In turn, "Netting" neutrophils may activate auto-reactive B cells leading to further antibody and immune complex formation. In this review we discuss on basic pathophysiology, epidemiology, genetics, clinical, laboratory and treatment updates of Felty's syndrome.
PubMed: 25614773
DOI: 10.2174/1874312901408010129 -
Journal of Medical Case Reports May 2021Felty syndrome is a rare manifestation of chronic rheumatoid arthritis in which patients develop extraarticular features of hepatosplenomegaly and neutropenia. The...
BACKGROUND
Felty syndrome is a rare manifestation of chronic rheumatoid arthritis in which patients develop extraarticular features of hepatosplenomegaly and neutropenia. The typical presentation of Felty syndrome is in Caucasians, females, and patients with long-standing rheumatoid arthritis of 10 or more years. This case report presents a patient with an early-onset and atypical demographic for Felty syndrome.
CASE PRESENTATION
Our patient is a 28-year-old African American woman with past medical history of rheumatoid arthritis diagnosed in 2017, asthma, pneumonia, anemia, and mild intellectual disability who was admitted to inpatient care with fever, chills, and right ear pain for 7 days. The patient's mother, also her caregiver, brought the patient to the hospital after symptoms of fever and ear pain failed to improve. Our patient was diagnosed with sepsis secondary to pneumonia and urinary tract infection. She had been admitted twice in the past year, both times with a diagnosis of pneumonia. During this visit in September 2019, it was discovered that the patient had leukopenia and neutropenia. Bone marrow biopsy revealed increased immature mononuclear cells with left shift and rare mature neutrophils. During the hospital course, the patient was provisionally diagnosed with Felty syndrome and treated with adalimumab and hydroxychloroquine for her rheumatoid arthritis. Her sepsis secondary to pneumonia and urinary tract infection was treated with ceftriaxone and doxycycline, which was later switched to cefepime because of positive blood and urine cultures for Pseudomonas aeruginosa. She was discharged with stable vital signs and is continuing to control her rheumatoid arthritis with adalimumab.
CONCLUSION
This case report details the clinical course of sepsis secondary to pneumonia and urinary tract infection in the setting of Felty syndrome. Our patient does not fit the conventional profile for presentation given her race, age, and the length of time following diagnosis of rheumatoid arthritis.
Topics: Adult; Arthritis, Rheumatoid; Felty Syndrome; Female; Humans; Neutropenia; Spleen; Splenomegaly
PubMed: 34039422
DOI: 10.1186/s13256-021-02802-9 -
British Medical Journal Nov 1971
Topics: Felty Syndrome; Female; Humans; Male; Middle Aged; Splenectomy
PubMed: 5124430
DOI: No ID Found -
Proceedings of the Royal Society of... Mar 1938
PubMed: 19991434
DOI: No ID Found