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Ann Biol Clin (Paris) 2018 Aug;76(4):429-434

Laboratoire de biologie clinique, CHU de Charleroi, Lodelinsart, Belgique.

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. Read More

Clin Dermatol 2018 Jul - Aug;36(4):533-550. Epub 2018 Apr 10.

Division of Dermatology, The University of Arizona College of Medicine, Tucson, Arizona, USA. Electronic address:

Systemic inflammatory disorders frequently involve the skin, and when cutaneous disease develops, such dermatologic manifestations may represent the initial sign of disease and may also provide valuable prognostic information about the underlying disorder. Familiarity with the various skin manifestations of systemic disease is therefore paramount and increases the likelihood of accurate diagnosis, which may facilitate the implementation of an appropriate treatment strategy. An improvement in quality of life and a reduction in the degree of morbidity may also be a realized benefit of accurate recognition of these skin signs. Read More

BMJ Case Rep 2018 Jul 11;2018. Epub 2018 Jul 11.

Department of Rheumatology, Mayo Clinic Arizona, Scottsdale, Arizona, USA.

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. Read More

Praxis (Bern 1994) 2018 Apr;107(8):463-466

1 Spitalzentrum Oberwallis, Spital Visp.

Clin Case Rep 2018 03 31;6(3):509-512. Epub 2018 Jan 31.

2nd Academic Department of Internal Medicine National and Kapodistrian University of Athens School of Medicine Hippokration General Hospital Athens Greece.

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 ) 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. Read More

G Ital Dermatol Venereol 2018 Apr 24;153(2):243-255. Epub 2018 Jan 24.

Unit of Dermatopathology, San Gallicano Dermatological Institute, Rome, Italy -

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. Read More

Hematology Am Soc Hematol Educ Program 2017 12;2017(1):181-186

University of Virginia, Charlottesville, VA.

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. Read More

Haematologica 2018 02 7;103(2):304-312. Epub 2017 Dec 7.

Hematology Research Unit Helsinki, University of Helsinki and Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center, Finland

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 and mutations were discovered in 30-40% of patients with large granular lymphocyte leukemia. Read More

Biomed Res Int 2017 13;2017:6307109. Epub 2017 Jul 13.

Department of Environmental & Occupational Health, Florida International University, Miami, FL, USA.

The overwhelming increase in the global incidence of obesity and its associated complications such as insulin resistance, atherosclerosis, pulmonary disease, and degenerative disorders including dementia constitutes a serious public health problem. The Inhibitor of DNA Binding/Differentiation-3 (ID3), a member of the ID family of transcriptional regulators, has been shown to play a role in adipogenesis and therefore ID3 may influence obesity and metabolic health in response to environmental factors. This review will highlight the current understanding of how ID3 may contribute to complex chronic diseases via metabolic perturbations. Read More

Scand J Rheumatol 2018 07 28;47(4):340-341. Epub 2017 Jul 28.

c Department of Radiology , National Cheng Kung University Hospital , Tainan , Taiwan.

Medsurg Nurs 2017 03;26(2):105-9, 118

Felty's syndrome is a triad of rheumatoid arthritis, splenomegaly, and neutropenia. This rare disorder is difficult to diagnose and produces many complications. The purpose of this descriptive qualitative case study was to provide a comprehensive, context-bound understanding of one patient's struggle with the condition. Read More

J Clin Rheumatol 2017 Jan;23(1):70-71

Department/Institution, Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University hospital Dubrava, University of Zagreb School of Medicine, Avenija Gojka Šuška 6, Zagreb, Croatia.

Pathol Res Pract 2016 Dec 17;212(12):1191-1193. Epub 2016 Oct 17.

Department of Pathology, Amiens University Hospital, France.

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. Read More

Neth J Med 2015 Nov;73(9):435-6

Department of Internal Medicine, Henry Ford Health System, Detroit, MI.

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. Read More

Reumatol Clin 2016 Jul-Aug;12(4):223-5. Epub 2015 Aug 24.

Servicio de Clínica Médica, Hospital Provincial del Centenario , Rosario, Santa Fe, Argentina.

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. Read More

Joint Bone Spine 2015 Jul 26;82(4):235-9. Epub 2015 Mar 26.

Service de médecine interne, hôpital du Haut-Lévêque, université de Bordeaux, 33604 Pessac, France; Département de rhumatologie, hôpital Lapeyronie, université de Montpellier 1, 34295 Montpellier cedex 5, France.

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. Read More

Acta Derm Venereol 2015 Jul;95(6):754-5

Department of Dermatology, Gifu University School of Medicine, 1-1 Yanagido, Gifu City 501-1194, Japan.

Joint Bone Spine 2015 May 23;82(3):196-9. Epub 2015 Jan 23.

Department of rheumatology, CHRU de Besançon, 2, Boulevard Fleming, 25030 Besançon, France. Electronic address:

Objective: Pseudo Felty's syndrome (PFS) is an uncommon syndrome occurring in Rheumatoid Arthritis and characterized by a monoclonal expansion of lymphocytes with neutropenia. Methotrexate is the first line recommended treatment. In case of incomplete response, cyclophosphamide may be used for hematological features but does not share the same efficacy on arthritis. Read More

J Clin Rheumatol 2015 Jan;21(1):53-5

Division of Rheumatology Department of Medicine Stony Brook University Medical Center Stony Brook, NY Long Island Regional Arthritis & Osteoporosis Care PC, Babylon Department of Medicine Mt Sinai School of Medicine, New York Department of Radiology Prohealth Care Associates Lake Success, NY.

Reumatol Clin 2015 Jul-Aug;11(4):227-31. Epub 2014 Nov 11.

Sección de Reumatología, Hospital J. M. Cullen, Santa Fe, Argentina.

We describe the case of a 51-year-old woman with a seropositive, erosive, and non-nodular rheumatoid arthritis of 15 year of evolution. The patient had poor compliance with medical visits and treatment. She came to the clinic with persistent pancytopenia and spleen and liver enlargement. Read More

J Clin Rheumatol 2014 Dec;20(8):440-1

From the *Commonwealth Medical College, Scranton; and †Arthritis Center, Department of Rheumatology, Susquehanna Health Medical Group, Williamsport, PA.

Blood Coagul Fibrinolysis 2015 Mar;26(2):211-3

aDepartment of Internal Medicine bDivision of Hematology and Oncology, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Large granular lymphocyte (LGL) leukemia is an indolent lymphoproliferative malignancy which dysregulates humoral immunity and underlies the myriad autoimmune phenomena. We describe a 62-year-old woman with Felty's syndrome who developed a severe bleeding diathesis. Laboratory evaluation demonstrated acquired inhibitors to both factor VIII (FVIII) and fibrinogen, likely secondary to T-cell LGL leukemia. Read More

J Clin Rheumatol 2014 Oct;20(7):398

Department of Physical Medicine and Rehabilitation Ankara University Medical School Altındağ, Ankara Turkey 06230 Department of Physical Medicine and Rehabilitation Ankara University Medical School Ankara, Turkey.

Eur J Rheumatol 2014 Sep 1;1(3):120-122. Epub 2014 Sep 1.

Department of Internal Medicine, Sakarya Training and Research Hospital, Sakarya, Turkey.

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. Read More

Atlas Oral Maxillofac Surg Clin North Am 2014 Sep;22(2):135-51

Division of Oral and Maxillofacial Pathology, School of Dentistry, University of Minnesota, 515 Delaware Street SE #16-116B, Minneapolis, MN 55455, USA. Electronic address:

Z Rheumatol 2014 Jun;73(5):465-6, 468

Felty’s syndrome is a rare variant of severe seropositive rheumatoid arthritis with neutropenia and splenomegaly. It is difficult to treat and associated with a poor prognosis due to the substantial risk of infections. This article presents the case of a patient with refractory disease who responded to rituximab with permanent normalization of neutrophil counts. Read More

Int J Rheum Dis 2014 Jan 2;17(1):122-4. Epub 2013 Jul 2.

Department of Rheumatology, Second Affiliated Hospital, Nanchang, China.

Ann Rheum Dis 2014 Mar 29;73(3):483-91. Epub 2013 Nov 29.

Program in Cellular and Molecular Medicine, Boston Children's Hospital and Harvard Medical School, , Boston, Massachusetts, USA.

Tolerance blocks the expression of autoantibodies, whereas autoimmunity promotes it. How tolerance breaks and autoantibody production begins thus are crucial questions for understanding and treatment of autoimmune diseases. Evidence implicates cell death and autoantigen modifications in the initiation of autoimmune reactions. Read More

Mod Rheumatol 2015 Sep 29;25(5):790-3. Epub 2015 Jul 29.

a Division of Immunology and Allergology, University Hospitals of Geneva , Switzerland.

Background: Rheumatoid arthritis (RA) is a risk factor for the development of Felty's syndrome and large granular lymphocyte (LGL) leukemia. Anti-cyclic citrullinated peptide (CCP) antibodies are considered highly specific for RA and are directed against various citrullinated antigens, including citrullinated fibrinogen. Anti-CCP antibodies may interfere with the detection of citrullinated proteins and their function. Read More

Rev Med Interne 2013 Sep 5;34(9):553-60. Epub 2013 Aug 5.

Service de médecine interne, hôpital du Haut Lévêque, avenue Magellan, 33604 Pessac, France.

Large granular lymphocyte leukemia (LGL) is a hematologic disorder characterized by a monoclonal expansion of large lymphocytes containing azurophilic granules with a T CD3(+)CD57(+) or Natural Killer (NK) CD3(-)CD56(+) phenotype. The World Health Organization (WHO) classification identifies three entities: the T LGL, the chronic lymphoproliferative disorder of NK-cells, and the aggressive NK-cell leukemia. T LGL and chronic lymphoproliferative disorder of NK-cells are indolent diseases frequently associated with cytopenias and a wide spectrum of auto-immune manifestations. Read More

Ann Pharmacother 2013 Apr 19;47(4):e19. Epub 2013 Mar 19.

Pharmacy Services, University of Alberta Hospital, Edmonton, Alberta, Canada.

Objective: To describe 2 recent cases of suspected immunoglobulin-mediated interference with the Beckman Coulter particle-enhanced turbidimetric inhibition immunoassay (PETINIA) used to measure vancomycin serum or plasma concentrations and to review the existing literature.

Case Summary: A 64-year-old woman with a history of multiple immune-related comorbidities received vancomycin for treatment of a prosthetic joint infection growing coagulase-negative Staphylococcus spp. A 33-year-old man with a history of Felty syndrome received vancomycin for the treatment of methicillin-resistant Staphylococcus aureus pneumonia. Read More

Ther Umsch 2013 Mar;70(3):157-62

Abteilung Hämatologie und Hämatologisches Zentrallabor, Luzerner Kantonsspital, Luzern.

The spleen functions as a filter of the circulating blood, removing aging or abnormal red blood cells, intraerythrocyte inclusions as well as foreign particals. As the spleen is composed of lymphocytic tissue, circulatory elements and mononuclear phagocytic cells it plays an important role in the nonspecific as well as the specific immune response. Additionally, the spleen serves as a reservoir for circulating blood cells, especially platelet sequestration by the spleen is well do cumented. Read More

Ann Hematol 2013 Jul 16;92(7):985-7. Epub 2013 Jan 16.

Clin Rheumatol 2013 May 5;32(5):701-4. Epub 2013 Jan 5.

B. Shine Department of Rheumatology, Rambam Health Care Campus and Technion, POB 9602, Haifa 31096, Israel.

Felty's syndrome (FS) is characterized by neutropenia and splenomegaly in patients with seropositive (RF+, anti-CCP+) rheumatoid arthritis (RA). As a result of neutropenia, affected persons are increasingly susceptible to infections. In the majority of patients, FS appears in the course of long-standing and well-established RA. Read More

Curr Rheumatol Rev 2013 ;9(1):34-44

III Department of Internal Medicine, Faculty of Medicine and Dentistry, Palacky University of Olomouc, I.P. Pavlova 6, 772 00 Olomouc, Czech Republic.

SLE is characterized by overproduction of various types of autoantibodies. Under certain circumstances, antibodies targeting some of the neoepitopes of the complement system can be seen. The most studied among antibodies directed against a component of the complement system is anti-C1q. Read More

J Postgrad Med 2012 Jul-Sep;58(3):217-20

Department of Nephrology, Bhopal Memorial Hospital and Research Centre, Bhopal, Madhya Pradesh, India.

This case describes a 42-year-old female with longstanding history of rheumatoid arthritis (RA) and Felty syndrome (FS). She presented with acute renal kidney failure, skin rash and hemoptysis. A clinical suspicion of small vessel vasculitis (SVV) was thought, serology was also positive for various markers of SVV. Read More

Clin Lymphoma Myeloma Leuk 2012 Dec 19;12(6):400-5. Epub 2012 Sep 19.

Department of Medicine, University of Connecticut Medical Center, Farmington, CT 06030-1235, USA.

Large granular lymphocyte (LGL) leukemia features a group of indolent lymphoproliferative diseases that display a strong association with various autoimmune conditions. Notwithstanding, these autoimmune conditions have not been comprehensively characterized or systematized to date. As a result, their clinical implications remain largely unknown. Read More

J Clin Rheumatol 2012 Aug;18(5):278

Pol Arch Med Wewn 2012 ;122(7-8):374-9

Department of Microbiology, Immunology and Biochemistry, University of Tennessee Health Sciences Center, Memphis, Tennessee, USA.

Felty's syndrome (FS) is a severe arthritic disorder that features chronic neutrophil activation and progresses to neutropenia and susceptibility to unabated infections. The life‑threatening manifestations of FS have focused the attention of clinical experimenters who have made persistent efforts to find new and effective therapies. This review highlights important milestones in the research on FS and draws attention to recent studies on the antigen specificity of antibodies present in patients' sera. Read More

Leg Med (Tokyo) 2012 Sep 4;14(5):246-8. Epub 2012 May 4.

Department of Forensic Medicine, Tokai University School of Medicine, Kanagawa, Japan.

Familial neglect was suspected when an older deceased female was found to have large decubitus ulcers and weight loss. Postmortem examinations including histopathology and bacterial culture revealed systemic Staphylococcus aureus infection as the cause of death. The victim might have exhibited Felty syndrome, which is characterized by complications of splenomegaly and neutropenia in the underlying rheumatoid arthritis. Read More

Joint Bone Spine 2012 Dec 7;79(6):624-5. Epub 2012 Mar 7.

Hôpital Lariboisière, Rheumatology Department, pôle appareil locomoteur, centre Viggo-Petersen, 2 rue Ambroise-Paré, Paris, France.

Felty's syndrome (FS) is a rare association of rheumatoid arthritis (RA), neutropenia and splenomegaly. Mechanisms of neutropenia in FS are unclear but involve both innate and humoral immunity, impaired granulopoiesis and decreased granulocyte half-life. Several treatments have been used without clear efficiency. Read More

Mil Med 2012 Jan;177(1):113-7

Section of Hematology/Oncology, Medical Service, Ralph H. Johnson Veterans Affairs Medical Center, Charleston, SC 29401, USA.

Acquired agranulocytosis is a rare, life-threatening disorder. The few known causes/associations usually are readily identifiable (e.g. Read More

Otol Neurotol 2012 Feb;33(2):258-66

University of California-Los Angeles, Division of Head and Neck Surgery, California 90095, USA.

Hypothesis: The histopathology of Sjogren's syndrome (SS) in the human inner ear correlates with mouse models of autoimmune inner ear disease.

Background: SS is an autoimmune disease in which 25% of patients have sensorineural hearing loss (SNHL). The inner ear histology in a SS mouse model has shown degeneration of the stria vascularis (SV) and immunoglobulin G deposition on the basement membrane of SV blood vessels. Read More

Semin Arthritis Rheum 2012 Apr 25;41(5):658-68. Epub 2011 Nov 25.

Department of Rheumatology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona, Spain.

Objective: To review and summarize the information available on the effectiveness and safety of biological therapies in refractory Felty's syndrome (FS).

Methods: We describe a case of FS with severe neutropenia and recurrent bacterial infections unresponsive to disease-modifying antirheumatic drug treatment and long-term administration with granulocyte colony-stimulating factor, in which treatment with rituximab (RTX) was useful and resulted in a sustained neutrophil response. Current evidence on the use of biological therapies in FS is also analyzed through a systematic review of the English-language literature, based on a PubMed search. Read More

Arthritis Rheum 2012 Apr 27;64(4):982-92. Epub 2011 Oct 27.

University of Tennessee Health Science Center, Memphis, TN 38163, USA.

Objective: To test the hypothesis that autoantigen modifications by peptidylarginine deiminase type 4 (PAD-4) increase immunoreactivity.

Methods: We assembled sera from patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Felty's syndrome (FS), and antineutrophil cytoplasmic antibody-associated vasculitides (AAVs), as well as sera from control subjects without autoimmune diseases. The sera were tested for binding to activated neutrophils, deiminated histones, and neutrophil extracellular chromatin traps (NETs). Read More

Curr Opin Hematol 2011 Jul;18(4):254-9

Department of Medicine, Penn State Hershey Cancer Institute, Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033-0850, USA.

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. Read More

Autoimmun Rev 2011 May 18;10(7):432-7. Epub 2011 Jan 18.

Department of Internal Medicine, Jamaica Hospital Medical Center, NY 11418, United States.

Autoimmune neutropenia, caused by neutrophil-specific autoantibodies is a common phenomenon in autoimmune disorders such as Felty's syndrome and systemic lupus erythematosus. Felty's syndrome is associated with neutropenia and splenomegaly in seropositive rheumatoid arthritis which can be severe and with recurrent bacterial infections. Neutropenia is also common in systemic lupus erythematosus and it is included in the current systemic lupus classification criteria. Read More

Clin Rheumatol 2011 Jun 19;30(6):843-7. Epub 2011 Jan 19.

Department of Rheumatology, Manisa Hospital, Manisa Devlet Hastanesi, Manisa, Turkey.

The study was designed to describe demographic, clinical, serological, and radiological characteristics of patients with rheumatoid arthritis (RA) followed-up by a single institution. One hundred sixty-five patients, diagnosed as RA using ACR classification criteria, and followed-up in the rheumatology clinic between December 2005 and January 2010, were enrolled in the study. Of the patients, 125 were female, and 40 were male. Read More

Rev Bras Reumatol 2010 Nov-Dec;50(6):710-3

Hospital Santa Casa de Belo Horizonte, Minas Gerais, Brazil.

Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. Read More

Dig Dis Sci 2011 Feb 4;56(2):295-302. Epub 2011 Jan 4.

Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.

Rheumatoid arthritis (RA), characterized by inflammation of the synovium and surrounding structures, has a prevalence of 0.5-1%. Rheumatoid vasculitis (RV) is an inflammatory condition of the small- and medium-sized vessels that affects up to 5% of patients with RA with intestinal involvement in 10-38% of these cases. Read More