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)

Two patients with rheumatoid arthritis without splenomegaly or Felty's syndrome developed spontaneous rupture of the spleen. The histological appearance was consistent with involvement of the splenic capsule by the rheumatoid process, and this may have predisposed to splenic rupture.
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PMID:Spontaneous rupture of the spleen in rheumatoid arthritis. 688 36

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.
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PMID:[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality]. 719 36

A 63 year-old female patient with rheumatoid arthritis and positive rheumatoid serology presented persistent neutropenia without spleen enlargement detectable by either physical or isotopic means. A pattern identical to that described for classical Felty's syndrome was found on determination of the IgG bound to granulocytes by inhibition of the hemolysis of sheep erythrocytes previously incubated with human IgG. Inhibition of hemolysis in the patient was 94 %, 63 %, and 32 % (according to the concentration of granulocytes per mm3), while in the healthy control it was 37.5 %, 30 %, and 0 %, respectively. In spite of the absence of spleen enlargement, the patient was diagnosed as suffering from Felty's syndrome without splenomegaly. The diagnostic and follow-up value of the measurement of IgG bound to granulocytes is emphasized.
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PMID:[Felty's syndrome without splenomegaly. A case report (author's transl)]. 720 85

Clinical parameters and laboratory studies including granulocyte-associated IgG were documented in 15 patients with Felty's syndrome treated by splenectomy. Five patients did not benefit from splenectomy, six responded partially, and four completely. Response to splenectomy could not be predicted from age, sex, splenomegaly, preoperative granulocyte count, platelet count, lymphocyte count, bone marrow lymphocytosis, or granulocyte bound IgG. In contrast, marked elevation in preoperative serum granulocyte binding IgG predicted response to splenectomy. Furthermore, a postoperative fall in serum granulocyte binding IgG was associated with response. Thus, one beneficial effect of splenectomy in some Felty's syndrome patients is reduction of serum granulocyte binding immunoglobulin.
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PMID:Felty's syndrome: effects of splenectomy upon granulocyte count and granulocyte-associated IgG. 723 95

Felty's syndrome is diagnosed when a patient shows both splenomegaly and leukocytopenia of various degree during the course of rheumatoid arthritis (RA). The accompanying immunologic abnormalities (e.g., antinuclear antibody, antiplatelet antibody, and hypocomplementemia) also characterize Felty's syndrome, but some authors may regard these abnormalities as a transitional form into overlap syndrome [RA + systemic lupus erythematosus (SLE)]. Here we reported a female case of Felty's syndrome who showed marked thrombocytopenia and severe hypocomplementemia. Thrombocytopenia had been refractory against several forms of therapies including high-dose methylprednisolone. Simultaneously, she had various autoantibodies (i.e., antiplatelet antibody, positive Coombs' test, antithyroglobulin antibody, antimicrosome antibody and anti-RNP antibody). Although she did not fulfill the ARA diagnostic criteria for SLE, the degree of thrombocytopenia as well as that of hypocomplementemia argued in favor of the overlap of SLE in this patient. Low-dose cyclosporin A (CsA) combined with small dose of prednisolone could increase both platelet count and level of complement. Notably, the titers of several autoantibodies dropped after CsA was started. These findings might suggest that CsA could normalize the underlying immunologic abnormalities in this patient. However, the disease activity of RA could not be decreased without a help of low-dose methotrexate.
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PMID:[A case of Felty's syndrome with marked thrombocytopenia and severe hypocomplementemia]. 755 58

Current treatment for Felty's syndrome, a triad of rheumatoid arthritis, splenomegaly and neutropenia, is often unsuccessful. Felty's syndrome may be related to decreased production of hematopoietic growth factors. We treated a patient with Felty's syndrome, profound neutropenia and a history of multiple complicated hospitalization for severe infections, with granulocyte colony stimulating factor (GCSF) for 18 months. After initiation of GCSF, the patient's neutrophil count has remained in the normal range for 18 months. After 2 easily treated infections at the start of therapy, she had only one episode of cellulitis occurring after 18 months when her GCSF dose was reduced to every 3rd day. She has been infection-free since then on every other day therapy. GCSF may be a cost effective longterm therapy for selected patients with Felty's syndrome and may reduce both patients' morbidity and overall medical costs.
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PMID:A prolonged use of granulocyte colony stimulating factor in Felty's syndrome. 769 67

A 64-year-old male patient with Felty's syndrome was treated with antibiotics, Plaquenil (hydroxychloroquine sulfate), and gold salts. In the fourth week of hospitalization, the patient died. Autopsy showed extensive bronchopneumonia, fibrous pleuritis, congestive splenomegaly, mild atherosclerosis, reactive lymphoid hyperplasia, congested passive liver, severe rheumatoid arthritis, and hypercellular bone marrow.
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PMID:Felty's syndrome: a case presentation. 812 Dec 59

In order to evaluate the place of the laparoscopic approach in splenectomy for haematological disease, the authors prospectively studied a series of 25 consecutive patients requiring splenectomy. There were 11 cases of thrombocytopenic purpura, 9 lymphomas, 2 cases of herediary spherocytosis, 1 Felty syndrome, 1 idiopathic myelofibrosis and 1 Hodgkin disease. Twelve patients (48%) underwent an immediate conventional procedure for huge splenomegaly (10), obesity (1), unavailability of video-equipment. Thirteen patients (52%) underwent a laparoscopic approach. Five of these operations were converted into a conventional approach for various reasons. In the other 8 patients, the spleen was completely released laparoscopically. In two of these 8 patients, the spleen was removed via a sub-pubic Pfannenstiel incision due toits volume. The last 6 spleens (24%) were removed in a plastic bag, corresponding to 5 cases of one thrombocytopenic purpura and one Hodgkin disease. None of these patients were obese. These results suggest that the laparoscopic approach is indicated in case of moderate splenemegaly in non-obese patients.
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PMID:[Place of celio-video-surgery in splenectomy for hematologic diseases]. 852 38

Felty syndrome, comprised of neutropenia, rheumatoid arthritis and splenomegaly, occurs in approximately 1% of patients with rheumatoid arthritis. Up to one third of these patients have an increased number of large granular lymphocytes. The usual immunophenotype of these cells is CD3+, CD8+, CD57+, T cell receptor (TCR) alpha beta. A patient with Felty syndrome and large granular lymphocytosis, who had an unusual immunophenotype CD3+, CD4-, CD8-, TCR gamma delta, is described. Her neutropenia responded to treatment with granulocyte colony stimulating factor (G-CSF), which was given in order to raise her neutrophil count prior to bilateral knee replacement surgery. Thus, Felty syndrome with large granular lymphocytosis is a heterogeneous condition, one in which TCR gamma delta large granular lymphocytosis may be found, and also shows a response to treatment with G-CSF.
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PMID:An unusual association of Felty syndrome and TCR gamma delta lymphocytosis. 865 18

Felty's syndrome is characterized by neutropenia, splenomegaly, and recurrent infection in patients with rheumatoid arthritis. We used recombinant granulocyte colony stimulating factor (rGCSF) in a patient with Felty's syndrome and recurrent sepsis. rGCSF induced a statistically significant increase in the patient's absolute neutrophil and total white blood cell counts. During 14 months of followup taking rGCSF, disseminated varicella zoster was the only infectious complication. Except mild thrombocytopenia and a transient flare of arthritis, no serious adverse effects occurred. rGCSF may be a safe and effective therapy for Felty's syndrome in selected patients.
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PMID:Resolution of the neutropenia of Felty's syndrome by longterm administration of recombinant granulocyte colony stimulating factor. 873 Jan 42


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