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)

The clinical and laboratory features of 72 patients with Felty's syndrome described within the last ten years have been compared with Felty's five original patients. Felty's syndrome appears to be a variant of rheumatoid arthritis with extra-articular manifestations in which leukopenia (usually due to neutropenia) and splenomegaly occur, although not always at the same time. Both are manifestations of the underlying disease process and are not necessarily otherwise related. The mechanism of the leukopenia is complex and abnormalities in leukocyte function appear to be as important as the leukopenia in predisposing patients with Felty's syndrome to infection. Functional abnormalities of the leukocytes in this syndrome are due in part to immune complex formation. Hypocomplementemia associated with this process may be another cause for the increased susceptibility to infection. It is proposed, therefore, that therapy in Felty's syndrome be directed at the underlying disease process, and gold salts and penicillamine should be considered for this purpose. Splenectomy should be reserved for specific situations, such as hemolytic anemia, severe thrombocytopenia, leg ulcers, and infections associated with profound leukopenia that are not responsive to medical therapy.
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PMID:Felty's syndrome: an analytical review. 33 Sep 14

Evidence is presented that Felty's syndrome (FS) is rare among black patients with rheumatoid arthritis (RA). All of 12 patients with FS seen at Parkland Memorial Hospital, Dallas, Texas betwen 1964 and 1978 were white. During this period 52% of patients admitted to the Parkland medical service were black and 31% of patients dischargd with a diagnosis of RA were black. The number of expected black cases of FS on the basis of the racial distribution of hospitalized patients with RA was 3.7 (P is less than 0.02 when the zero incidence in blacks was compared with the expected incidence). All 7 cases of FS observed at Charity Hospital, New Orleans, Louisiana between 1968 and 1978 were also white. During this period, 65% of patients discharged with a diagnosis of RA were black, and the number of expected black cases of FS was 4.5 (P is less than 0.001). These findings suggest a genetic basis for the development of leukopenia and splenomegaly in RA patients.
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PMID:The rarity of Felty's syndrome in blacks. 47 75

Neutrophil marrow cellularity was determined in 14 neutropenic patients with rheumatoid arthritis (RA) from measurements of neutrophil-normoblast ratios in marrow biopsies and ferrokinetic estimates of marrow normoblasts. A marrow profile was developed for each patient comprising the numbers of promyelocytes and myelocytes, of metamyelocytes and bands, and of segmented neutrophils in whole marrow. In each case a maturation ratio was calculated by dividing the number of metamyelocytes and bands by the number of promyelocytes and myelocytes. The physiologic marrow response to loss of neutrophils from circulation was assumed to be an increase in promyelocytes and myelocytes due to proliferation and influx, a reduction in segmented cells due to early release, and a normal maturation ratio. The results were interpreted in the light of the 95% confidence limits for data previously obtained from 13 normal subjects: in patients with neutropenia reduced or basal numbers of promyelocytes and myelocytes were interpreted as absence of the anticipated proliferative response; increased numbers of marrow segmented cells were attributed to failure of release; a low maturation ratio was assessed to reflect intramedullary cell loss. The pattern in two patients with Felty's syndrome was consistent with a physiological response to neutrophil destruction. The other 12 patients had neutrophil marrow abnormalities. Seven patients with Felty's syndrome and four patients without splenomegaly had absolute or relative hypoplasia of neutrophil marrow or low maturation ratios. One patient with a normal spleen size had an increased number of marrow segmented cells yet failed to mobilize cells normally in response to dialysis coil-activation of C3. Abnormalities of neutrophil marrow may contribute to neutropenia in RA irrespective of the presence of splenomegaly. Recognition of neutrophil marrow abnormalities in these patients may be of value in prognosis and management.
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PMID:Neutrophil marrow profiles in patients with rheumatoid arthritis and neutropenia. 52 42

A reaction of the RES with its organs (lymph nodes, spleen, bone marrow and Kupffer's cells) is not uncommon in rheumatoid arthritis. Although these manifestations are pronounced in Felty's syndrome, reviewing articles about Felty's do not mention liver manifestations. This paper summarises the publications about liver findings in Felty's syndrome. Hepatomegaly, abnormal bromsulfalein tests, raised alkaline phosphatase and transaminases have been stated in many case reports. Among 34 patients, the frequency of hepatomegaly was 68%, of abnormal bromsulfalein tests 27%, of alkaline phosphatase 23% and of transaminases 18%, respectively (52). Alkaline phosphatase and transaminases were raised in almost all of 12 patients (3). The histological correspondence is an infiltration with lymphocytes of sinusoids and portal fields, a portal fibrosis and occasionally a cirrhosis. These histological abnormalities, as well as enlarged lymph nodes and splenomegaly, have to be considered as organic manifestations of rheumatoid arthritis. If the nodular regenerative hyperplasia of the liver, which has been reported also after use of contraceptives, is a manifestation of Felty's syndrome, remains unresolved.
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PMID:[Liver findings in Felty's syndrome. A review]. 69 99

A quantitative antiglobulin consumption technique was used to measure immunoglobulin G (IgG) present on human granulocytes. Granulocytes from 50 normal subjects had less than 20 X 10(-14) g IgG per cell. Patients with granulocytopenia due to bone-marrow failure, patients with IgG multiple myeloma, patients with splenomegaly, and patients with rheumatoid arthritis without granulocytopenia had granulocyte-bound IgG within the range of normal. Four patients with rheumatoid arthritis, splenomegaly, and severe granulocytopenia (Felty's syndrome) had granulocyte-bound IgG between 30 and 220 X 10(-14) g IgG per cell. One of these patients underwent splenectomy, after which his granulocyte-bound IgG fell to normal. Seven additional patients with Felty's syndrome who had previously undergone splenectomy had normal levels of granulocyte-bound IgG. Thus quantitation of granulocyte-bound IgG appears to be useful in defining patients with immunologically mediated granulocytopenia. Studies of patients with Felty's syndrome who have undergone splenectomy suggest that the spleen may produce this neutrophile-bound immunoglobulin.
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PMID:Felty's syndrome: granulocyte-bound immunoglobulin G and splenectomy. 97 Jul 68

To evaluate the effectiveness of splenectomy in the treatment of Felty's syndrome (association of rheumatoid arthritis, leukopenia, and splenomegaly), such experience from 1968 to 1972 at the University of Alabama Medical Center in Birmingham was analyzed. There were five patients with Felty's syndrome who underwent splenectomy. In all five patients, there was no operative morbidity, no blood transfusions were required, and leukopenia and susceptibility to infection were greatly improved; neutropenia disappeared in all but one patient. Splenectomy appears to benefit most patients with Felty's syndrome.
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PMID:Role of splenectomy in Felty's syndrome. 108 Mar 62

A case of upper gastrointestinal tract hemorrhage secondary to esophageal varices in a patient with Felty's syndrome prompted a review of the pathogenesis and treatment of this condition. Six previously reported cases of this association were found. The clinical picture is that of long-standing rheumatoid arthritis with severe articular and extraarticular manifestations including splenomegaly, depression of the blood elements, mild liver function abnormalities, portal hypertension without cirrhosis or portal vein obstruction, an elevated splenic blood flow, and a reduction in portal hypertension by simple splenectomy. The presence of portal hypertension with varices may be another indication of splenectomy in patients with Felty's syndrome.
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PMID:Esophageal varices in Felty's syndrome: A case report and review of the literature. 108 37

The clinical course of a patient with Felty's syndrome is described. This patient was unusual because during a 3-year period of splenomegaly and leukopenia she did not develop repeated infections, leg ulcers, or other complications of Felty's syndrome. Then a spontaneous remission began. During the subsequent 6 years, neither symptoms nor signs of Felty's syndrome have recurred.
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PMID:Spontaneous remission of Felty's syndrome. 119 53

A female patient simultaneously developed hematologic evidence of hairy cell leukemia and marked but short-lived inflammatory involvement of a number of joints. Both these groups of symptoms resolved simultaneously and rapidly under alpha-2 interferon therapy. This course suggests that the arthritis was a rheumatologic manifestation of the hematologic disease. The concomitant occurrence in this patient of arthritis, splenomegaly and leukopenia was suggestive of Felty syndrome: these two conditions need to be differentiated.
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PMID:[Polyarthritis revealing hairy cell leukemia]. 130 95

A 72-year-old woman presented with a diffuse infiltrating soft tissue mass involving the entire right orbit. She had a pre-existing phthisis of the right eye secondary to retinal detachment, and had developed painless proptosis on the same side. Her medical history included rheumatoid arthritis, leucopenia, and an enlarged spleen, characteristics consistent with the diagnosis of Felty's syndrome. Risk of lymphoma is thought to be higher in patients with established connective tissue disorders. The case described herein, to our knowledge, represents the first report of an association between Felty's syndrome and orbital lymphoma. The symptoms of Felty's syndrome in our patient were initially attributed to spread of a malignant process involving the orbit.
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PMID:Orbital lymphoma in a patient with Felty's syndrome. 154 May 65


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