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)

We describe a case of Felty's syndrome with persistent severe neutropenia below 200 granulocytes/mm3, splenomegaly and repeated infections. The patient did not respond to treatment with intramuscular gold salts and lithium carbonate. After 2 months of oral methotrexate administration, 7.5 mg weekly, clinical improvement was notable: she remained afebrile, neutropenia disappeared and splenomegaly regressed. This clinical and laboratory improvement persisted 5 months later. Moreover, accidental discontinuance of the drug and later readministration supported the evidence that the improvement was due to methotrexate.
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PMID:Felty's syndrome: response to low dose oral methotrexate. 185 26

The proliferation of large granulated T lymphocytes (LGL) with neutropenia and splenomegaly can be observed in patients with rheumatoid arthritis (RA), in a similar way to Felty's syndrome. We report a female with long standing AR, xerophthalmia, xerostomia, neutropenia and LGL lymphocytosis. The phenotype of the latter was CD3+, CD8+, HNK1+. She was treated with methotrexate and corticosteroids. The genetic rearrangement study did not show monoclonality. Although LGL lymphocytosis can infiltrate several organs, salivary glands infiltration has not been reported. The development of a sicca syndrome can be a feature of LGL lymphocytosis and induce diagnostic mistakes in RA.
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PMID:[Sicca syndrome caused by large granulated lymphocytes in rheumatoid arthritis]. 262 75

The patient is a 44-year-old female case of RA having a history of 10 years after onset, being admitted due to fever accompanied by granulocytopenia. By laboratory findings, WBC was 1,900/mm3, CRP 5+, RA 2+, RAHA 80 times, LE cells positive, antinuclear antibody strongly positive, granulocyte-specific antinuclear antibodies positive, and granulocyte specific cytotoxicity test strongly positive. Examination of bone marrow smears revealed, the cell numbers tended to decrease in the neutrophil maturity phase. The case was diagnosed as Felty's syndrome because of the obvious splenomegaly and leukopenia. Administration of 30 mg/day of prednisolone was not sufficient in effect, and plasmapheresis was conducted concomitantly. As a result, the granulocyte count increased and the granulocyte cytotoxicity titer was lowered. It is hence suggested that the antigranulocyte antibodies are responsible for the mechanism of granulocytopenia in this case, and the possibility of promotion of destruction in the periphery was inferred, and it is accordingly considered useful to combine administration of small dose of adrenocortical steroid preparation with plasmapheresis for the treatment of this disease.
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PMID:[A case of antigranulocyte antibody positive Felty's syndrome and its efficacy of plasmapheresis]. 278 58

Three cases of severe Felty's syndrome (neutrophils 80/mm3 in 2 cases and 800/mm3 in one) with enlarged spleen (1 case) and leg ulcer (2 cases) were treated with high doses of corticosteroids (methylprednisolone 1 g by intravenous infusion over 3 hours, on 3 consecutive days). In all 3 patients a dramatic result was obtained, the granulocytes returning to normal values in 24 to 48 hours. In 2 cases, an early relapse (1 month) required a second course in high dosage; after a follow-up of 36 and 8 months respectively, the number of granulocytes remains stable under prednisone 5 to 10 mg/day. The third patient was improved by one single course of infusions and remains stable after 18 months. It must be noted that one patient had previously been treated with plasma exchange and another with corticosteroids without any significant result.
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PMID:[Treatment of Felty's syndrome accompanied by agranulocytosis using high-dose corticoids. 3 cases]. 295 52

Felty's syndrome (FS) refers to the occurrence of rheumatoid arthritis, splenomegaly, and neutropenia. A subset of these patients has recently been described with a chronic T cell leukemia of large granular lymphocytes (LGCL). To examine the spectrum of lymphocyte abnormalities in FS and LGCL, we examined phenotypic and genotypic properties of lymphocytes from eight FS patients. In two of these FS patients, we observed an elevated proportion of T cells with an unusual phenotype (CD3+/Leu-7+/Leu-8-/CR3+) (46 +/- 5% of mononuclear cells). The FS lymphocytes had large granular morphology on Wright-Giemsa stain and were active in antibody-dependent cellular cytotoxic activity. This phenotype, morphology, and activity was similar to LGCL patients except that the latter T cells additionally expressed the Fc-IgG receptor recognized by monoclonal antibody Leu-11 (CD 15). In the remaining six FS patients, the proportion of CD3+/Leu-7+/CR 3+ T cells was only 10 +/- 8%, which was not significantly different from age-matched normal subjects (6.6 +/- 2.2%). To determine the clonality of T lymphocytes in FS and LGCL, we examined DNA for rearrangements of the T cell antigen receptor beta-chain (Ti beta) and gamma-chain (Ti gamma) genes by using Southern blotting techniques. We found a clonal rearrangement of the Ti beta 1 and Ti gamma genes in both LGCL patients. In contrast, no clonal rearrangements of Ti beta or Ti gamma genes were detected in lymphocytes from the FS patients. These results indicate that FS patients are heterogeneous in their phenotype and that one subset exhibits polyclonal expansion of an unusual lymphocyte subset.
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PMID:Comparison of T cell receptor gene rearrangements in patients with large granular T cell leukemia and Felty's syndrome. 310 95

Some patients with chronically elevated large granular lymphocyte (LGL) numbers have rheumatoid arthritis (RA). Since these patients also may have neutropenia and splenomegaly, their symptoms resemble those of patients diagnosed as having Felty's syndrome (FS). We studied the immunophenotypic and genotypic characteristics of mononuclear cells from patients with RA and neutropenia to better determine the extent of heterogeneity in this condition. Four patients had markedly increased numbers of LGLs, which expressed HNK-1 antigen and IgG Fc receptors. In contrast, the remaining 8 patients, who had FS, had normal LGL counts, and surface marker studies showed normal numbers of HNK-1 and IgG Fc receptor positive cells. Clonal rearrangement of the T cell receptor beta chain gene was demonstrated in all 4 patients with excess LGLs, whereas a germline configuration of this gene was present in all 6 FS patients in whom this was studied. These results suggest that there are diverse groups among patients with RA and neutropenia. Since prognosis may differ, it is important to recognize that some patients who are considered to have Felty's syndrome may have a clonal proliferation of LGLs.
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PMID:Clonal proliferation of large granular lymphocytes in rheumatoid arthritis. 334 30

A 62-year-old female patient with splenomegaly, severe granulocytopenia, recurrent infections and negative rheumatoid factor test initially had no clinical evidence of rheumatoid arthritis. Leucopenia responded to splenectomy and did not recur during 7 years follow-up. Symmetrical metacarpophalangeal joint-swelling developed 9 months after 7 years of radiographic destruction. This case emphasizes that arthritis may occasionally be a late and minor manifestation of Felty's syndrome.
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PMID:[Incomplete Felty syndrome with manifestation of arthritis following a long-standing course of the disease]. 336 47

The authors report 18 cases of Felty's syndrome followed, in an average, for 5 years (1 to 12 years). There were 3 deaths, 9 patients are in complete remission, 6 others still show signs of the disease: splenomegaly (4 cases), leucopenia (1 case) and only one complete Felty's syndrome. Steroid therapy has proved to be effective regardless of the mechanism of the neutropenia specified in 9 cases by an isotopic study. Prognosis and infectious risk are difficult to determine but the overall course was rather favorable.
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PMID:[Mechanism of Felty's syndrome and long-term course]. 337 72

The Tc-99m sulfur colloid liver-spleen scintigrams of nine patients with Felty's syndrome (a triad of rheumatoid arthritis, splenomegaly, and neutropenia) were reviewed. The characteristic scintigraphic findings include (1) moderate or severe splenomegaly, (2) the reversal of liver-to-spleen uptake ratio despite normal liver function tests, and (3) virtually no visualization of the bone marrow uptake and no pulmonary radiocolloid sequestration. In a late stage of cirrhosis of the liver, moderate-to-severe splenomegaly with the reversal of liver-to-spleen uptake ratio almost always accompanies abnormal liver function, and sometimes there may be associated pulmonary radiocolloid uptake. It was therefore concluded that in a proper clinical setting, a radiocolloid scintigraph may differentiate between Felty's syndrome and cirrhosis of the liver in a late stage.
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PMID:Radiocolloid scintigraphy in Felty's syndrome. 348 13

In an adult man the spleen serves two physiologic functions. It is an organ of the immune system, the second function is the sequestration and removal of normal and abnormal blood cells. Most diseases of spleen are associated with enlargement of the organ. Causes of splenomegaly are liver diseases with portal hypertension, infections, malignant leukemias or lymphomas often combined with lymph node enlargement, collagen vascular diseases or Felty's syndrome, chronic hemolytic syndromes and infiltrative diseases such as Gaucher's disease. Some diseases associated with splenic enlargement respond to splenectomy.
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PMID:[Internal medicine diagnosis of splenic diseases]. 357 92


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