Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Felty's syndrome is regarded as a severe variant of rheumatoid arthritis (RA) that develops in less than 1% of patients with RA. It consists of a triad of RA, splenomegaly, and leukopenia, which tends to develop after a long course of RA. Treatment of neutropenia is mainly comprised of disease-modifying antirheumatic drugs including methotrexate, hydroxychloroquine, auronofin, penicillamine, glucocorticoids, and granulocyte monocyte colony stimulating factor. Recently, there has been a growing interest in the biologic agent rituximab in the treatment of Felty's syndrome. To our knowledge, only one previous case of rituximab being beneficial in the treatment of Felty's syndrome has been reported. We report the case of a 60-year-old man with Felty's syndrome in whom treatment with rituximab led to a sustained neutrophil response and marked symptomatic improvement in the form of decrease in the size of rheumatoid nodules and better pain control.
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PMID:Rituximab is useful in the treatment of Felty's syndrome. 1864 32

T cell large granular lymphocyte leukemia (T-LGL) is a disease characterized by clonal expansion of cytotoxic T cells (CTLs). It generally follows an indolent course and is notable for an association with chronic inflammation, neutropenia and rheumatoid arthritis (RA). We present herein a case of a patient with rheumatoid arthritis (RA), neutropenia, large granular lymphocytosis, and an expanded clonal population of peripheral blood CD3(+)CD8(+)TCRalphabeta CTLs, consistent with the diagnosis of T-LGL. T-LGL is part of a spectrum of large granular lymphocytic (LGL) disorders, which includes the more common indolent variety of this disease (as illustrated by the case herein), an aggressive but rare form of this leukemia, natural killer (NK) cell LGL leukemia, Felty's syndrome (FS), and chronic large granular lymphocytosis. T-LGL appears to be a relatively rare disease, but the true prevalence is not known. FS occurs in less than 1% of patients with RA and is typically defined by the triad of destructive arthritis, neutropenia, and variable splenomegaly. A subset of patients with FS will demonstrate polyclonal expansion of LGLs, implying a relationship between proliferation of LGLs and the mechanisms of neutropenia. Thus, T-LGL leukemia and FS with LGL expansion in the setting of RA is classically distinguished by the clonality of the CTL population, with monoclonality in T-LGL and polyclonality in FS. Despite this difference, T-LGL and FS are often similar in their clinical and biological behavior. Both may respond to immunosuppressive therapy, and pursue a smoldering course typical of a chronic inflammatory disease.
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PMID:T cell large granular lymphocyte leukemia associated with rheumatoid arthritis and neutropenia. 1939 80

Hairy-cell leukemia is a chronic B-cell malignancy seen in adults. The presenting manifestations consist of splenomegaly, pancytopenia, and characteristic monocyte depletion. The presence in peripheral blood or bone marrow of hairy cells exhibiting the CD19(+) CD20(+) CD25(+) CD11c(+) phenotype establishes the diagnosis. Rarely, patients present with inaugural joint manifestations related either to the hematological malignancy or to immune dysfunction. The resulting polymorphic polyarticular symptoms may cause diagnostic wanderings. Monocytopenia is a valuable diagnostic clue. The identification of hairy cells in the joint fluid establishes the diagnosis of leukemia-related arthritis. The treatment rests on purine analogs. One of the main differential diagnoses is Felty's syndrome, which combines rheumatoid arthritis, splenomegaly, and neutropenia. Felty's syndrome is usually caused by T-cell lymphoproliferative disorders. Among 27 patients with hairy-cell leukemia managed at our institution, 1 presented with joint manifestations. We describe this case.
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PMID:Hairy-cell leukemia with inaugural joint manifestations. 1954 26

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56(-) natural killer-cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56(-) LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.
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PMID:Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. 1996 15

Wrist involvement in rheumatoid arthritis (RA) is common. Within 2 years of diagnosis, more than half of patients will have wrist pain, and more than 90% will have wrist disease by 10 years. Although wrist involvement is generally thought to be less disabling than RA of the fingers and hand, it can be a significant cause of pain and disability. Severe disease with bony destruction and synovitis in the wrist can also result in soft-tissue problems including tendon ruptures. In addition to musculoskeletal involvement, systemic manifestations of RA can occur. Felty syndrome can result in a low white blood count and splenomegaly in association with RA. New generation, disease-modifying pharmacologic agents offer promise in controlling the disease progression. Surgical treatments for the diseased wrist are aimed at relieving pain and restoring function. Common procedures include: synovectomy and tenosynovectomy, tendon reconstruction, distal ulnar resection and/or distal radioulnar joint reconstruction, partial and full wrist arthrodesis, and total wrist arthroplasty.
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PMID:Current concepts and treatment for the rheumatoid wrist. 2117 1

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. Clinically apparent RV of the gastrointestinal (GI) tract, while rare, is often catastrophic, resulting in ischemic ulcers and bowel infarction. Vasculitis of the colon may present as pancolitis clinically similar to ulcerative colitis. Rectal biopsies that include submucosal vessels are positive for vasculitis in up to 40% of cases. Abnormal esophageal motility in RA may result in heartburn and dysphagia. Chronic atrophic gastritis may be associated with hypergastrinemia and hypo- or achlorhydria, promoting small bowel bacterial overgrowth. RA is the most common cause of secondary amyloidosis with GI symptoms in 22% of affected patients. Although amyloid is usually found in the liver, it is rarely evident clinically. Felty's syndrome occurs in less than 1% of patients with RA and is characterized by neutropenia and splenomegaly. The liver may be involved with portal fibrosis or nodular regenerative hyperplasia. Liver histology is abnormal in 92% of RA patients at autopsy, although the changes are usually mild without associated hepatomegaly. Drug-induced liver disease may occur with aspirin, sulfasalazine, and methotrexate. Significant liver damage is rare if the drug is discontinued or the patient is properly monitored. RA can affect both the GI tract and the liver; changes are usually mild except with RV.
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PMID:Gastrointestinal and hepatic manifestations of rheumatoid arthritis. 2120 2

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. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.
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PMID:Felty's syndrome and Kala-azar: a challenge for the rheumatologist. 2124 9

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. The pathobiology of the autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus is complex, and it could be a major cause of morbidity and mortality due to increased risk of sepsis. Treatment should be individualized on the basis of patient's clinical situation, and prevention or treatment of the infection. Recombinant human granulocyte colony-stimulating factor is a safe and effective therapeutic modality in management of autoimmune neutropenia associated with Felty's syndrome and systemic lupus erythematosus, which stimulates neutrophil production. There is a slight increased risk of exacerbation of the underlying autoimmune disorder, and recombinant human granulocyte colony-stimulating factor dose and frequency should be adjusted at the lowest effective dose.
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PMID:Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus. 2125 89

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. We report a patient with FS efficiently treated with rituximab (RTX), the monoclonal anti-CD20 antibody. A literature review of FS treated with RTX was performed.
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PMID:One case of Felty's syndrome efficiently treated with rituximab. 2240 85

Large granular lymphocyte leukemia is a rare entity belonging to same spectrum of diseases than Felty's syndrome, which might occur in patients with long-standing rheumatoid arthritis. It is clinically characterized by persistent neutropenia and recurrent bacterial infections associated with the presence in both peripheral blood and bone marrow of clonal expansion of atypic lymphocytes with a cytotoxic T cell phenotype, or less frequently an NK-cell phenotype, as well as splenomegaly. It is more frequently diagnosed in seropositive rheumatoid arthritis, with significant structural damage, extra-articular manifestations and persistently elevated values of ESR, despite them havubg low inflammatory joint activity. We report the case of a 70 year old male with a long-standing rheumatoid arthritis, who developed septic shock secondary to prosthetic hip infection by Salmonella spp. He showed persistent neutropenia, and an aberrant monoclonal T cell population was detected in both peripheral blood and bone marrow, consistent with large granular lymphocyte leukemia.
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PMID:Large granular lymphocyte leukemia as a complication of rheumatoid arthritis. 2248 60


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