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 first case of Felty's syndrome to be reported in a Nigerian, a fifty five-year-old woman who developed splenomegaly and leukopenia during the course of strongly seropositive rheumatoid arthritis of ten years duration is presented. her arthritis had lasted for about ten years, but she did not have the severe deforming disease known to be associated with Felty's syndrome. Radiologically there were no erosions and subcutaneous nodules were absent. She had a positive granulocyte specific anti-nuclear factor, cryoglobulins containing IgA and IgM and her polymorph-nuclear granulocytes showed evidence of impaired killing of staphylococci.
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PMID:Felty's syndrome in a Nigerian. 175 Jan 23

Felty's syndrome, consisting of rheumatoid arthritis, leukopenia, and splenomegaly, has been recognized as a distinct clinical entity for more than 60 years. Clinical and laboratory manifestations of the condition are reviewed. The major sources of morbidity and mortality remain recurrent local and systemic infections. Immunogenetic analysis shows a strong association with HLA-DR4, in addition to DQ beta 3b and C4B null allele. Potential mechanisms of neutropenia are contrasted, including impaired granulopoiesis and neutrophil-immune complex interactions. Lithium carbonate and splenectomy may have a role in the treatment of fulminant disease. Maintenance therapy should be directed at control of the underlying inflammatory arthropathy. A syndrome of proliferation of large granular lymphocytes and neutropenia, associated with rheumatoid arthritis in 23% to 39% of cases, has been described recently. Cases of "pseudo-Felty's" syndrome are often confused with traditional Felty's syndrome, which has twice the prevalence. The clinical and laboratory distinctions between these two conditions are elaborated.
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PMID:Felty's and pseudo-Felty's syndromes. 178 50

Two cases of Richter's syndrome are reported (in a 62 and 64 years old man) consistent with the appearance of B cell lymphoma of high malignancy in the course of CLL (low malignancy B cell lymphoma). In one patient, after 8-, and in the other one--after 53 months since the diagnosis of CLL, there was rapid clinical deterioration with lymphadenopathy, hepato- splenomegaly, fever and progressive cachexia, anemia and thrombocytopenia and leukopenia, unrelated to treatment. Both patients died, 4 and 3 months respectively, since the appearance of these symptoms. In the first cases Richter's syndrome was diagnosed histopathologically from the autopsy material. In the liver, spleen, adrenals and bone marrow, in addition to the characteristic infiltrates of CLL (small lymphocytes) there were areas of large cell proliferation consistent with high malignancy lymphoma. In the other case, the infiltrates of large cell lymphoma were found in the gall bladder removed because of acute cholecystitis, and in the lymph node from the hepatic hilar area. Immunocytochemical studies performed on the biopsy material indicated that the neoplastic cells had markers of B lymphocytes and cytoplasmic IgM kappa, as lymphocytes of CLL. In patients with CLL, who display rapid clinical deterioration and general symptoms with cachexia, the possibility of Richter's syndrome should be considered, and appropriate morphological studies performed.
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PMID:[2 cases of Richter's syndrome]. 182 59

Felty's syndrome (FS) consists of the triad: rheumatoid arthritis (RA), leukopenia and splenomegaly. FS occurs in approximately 1% of patients with RA. In this syndrome, the risk of infection is increased and anaemia, thrombocytopenia and cutaneous ulcers are more frequently observed. The literature is reviewed on the basis of a case history. The pathogenesis is unknown but is probably multifactorial. Cell antibodies, increased occurrence of immune complexes, inhibited neutrophil production, altered neutrophil distribution and reduced neutrophil function have been observed. The main indication for treatment is present if the patient has severe neutropenia (less than 0.1 x 10(9)/l) and repeated infections. Various methods of treatment are available. The most important are: gold, low-dose methotrexate, lithium, methylprednisolone pulse therapy, penicillamine and splenectomy. According to the literature, conventional steroid treatment cannot be recommended.
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PMID:[Felty's syndrome]. 186 80

Combined total body irradiation (TBI) and Prednimustine were prospectively evaluated in 30 patients affected either with chronic lymphocytic leukemia (CLL) or with low-grade non-Hodgkin's lymphoma (NHL) eleven patients were previously treated. Between January 1984 and May 1987, 20 evaluable patients with CLL, median age 66 years (range 43-82), classified according to Rai (4 in stage I, 10 in stage II, 4 in stage III, 2 in stage IV) and 10 evaluable patients with NHL low-grade malignancy according to the Working Formulation, Stages III and IV, median age 54 years (range 32-71) were treated using a 6 MeV Linear Accelerator, applying two opposite alternating fields including total body, with a fraction of 15 cGy, 2 fractions weekly (3-day interval) for a total dose of 150 cGy given over 5 weeks. Prednimustine (100 mg/m2, orally, for 5 consecutive days, every 3-4 weeks, for 6-9 courses) was administered 2 months after TBI treatment, as consolidation therapy. By May 1989, a total of 85% hematological responses (defined as normalization of the differential white cell count, of the total blood cell count and of bone marrow infiltration) were obtained after combined treatment in CLL patients; moreover 3 CR (according to the WHO criteria), 75% with splenomegaly reduction and 40% with lymphadenopathy reduction were seen. Ninety percent objective responses (5 CR and 4 PR) were observed in the NHL patients, with 50% having splenomegaly reduction and 67% lymphadenopathy reduction. The median response time in the two groups was, respectively, 14 and 23 months. The overall toxicity (WHO grades 1,2,3,4) after combined treatment was 65% and 70% in the two patient groups. WHO grade III toxicity, completely reversible, was verified in only 16.6% of the cases; all cases, except one, were previously treated. Additionally, 1 toxic death (grade IV thrombocytopenia and leukopenia) was observed in a heavily pretreated patient affected with CLL after TBI alone. Prednimustine regimen was generally well tolerated. The high response rate and acceptable toxicity, confirms the feasibility and the usefulness of TBI in the context of a combined treatment for CLL and low-grade NHL patients. However in order to further reduce the severe toxic side effects, observed in one patient, white blood cells and platelet count should be plotted and monitored carefully, particularly in pretreated patients.
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PMID:Feasibility of total body irradiation in chronic lymphocytic leukemia and low-grade non-Hodgkin's lymphomas. 188 46

The medical records of 370 patients treated for typhoid fever between 1986 and 1988 at the Communicable Disease Centre, Singapore, were reviewed. The disease was generally mild. There was no mortality. Fever was found in 98.4% of patients on admission and diarrhoea in 21%. Cough was predominantly a symptom of children and occurred in 7.1% of patients aged below 15 years. Other symptoms were uncommon. Hepatomegaly was found in 71% and splenomegaly in 47%. Leucopenia was not a helpful diagnostic marker. Chloramphenicol was the drug of choice. The relapse rate was 5.4% and the convalescent and temporary carrier rates 11.6%. The risk of developing the carrier state was significantly higher among patients who were afebrile on admission compared with those who were febrile (P less than 0.001); it was also higher in patients treated with ampicillin as compared to those treated with chloramphenicol (P less than 0.001, chi 2 = 22.7, odds ratio = 5.25, 95% confidence limits: 2.46 and 11.29). The role of ampicillin as a first line treatment for acute typhoid fever may need further re-evaluation.
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PMID:Typhoid fever in Singapore: a review of 370 cases. 194 16

To evaluate the efficacy of recombinant murine granulocyte-macrophage colony-stimulating factor (rGM-CSF) in attenuating the myelosuppression associated with chemotherapy, the effects of 100 and 300 ng rGM-CSF, administered twice daily by intraperitoneal injection for 6 consecutive days to mice 24 hours after a dose of 200 mg/kg cyclophosphamide, were measured. Six days after the initial injection of rGM-CSF, a significant increase occurred in the absolute myeloid count compared to that of vehicle-treated animals. The difference was most pronounced on day 7, attaining levels of 327% and 428% of the control; these increases slowly declined to that of the control level by day 19. No significant effect was produced by rGM-CSF on the packed red cell volume or on the platelet count. Furthermore, the administration of rGM-CSF did not alter bone marrow cellularity or increase the number of marrow-derived hematopoietic stem cells. In contrast, a significant splenomegaly occurred, starting on day 6 and continuing until day 17. This was characterized by a pronounced increase in splenic-derived granulocyte (CFU-G), granulocyte-macrophage (CFU-GM), macrophage (CFU-M), megakaryocyte (CFU-MK), and erythroid (BFU-E, CFU-E) stem cells. The increases occurred between days 6 and 9 following the initial administration of rGM-CSF. These findings indicated that the administration of rGM-CSF to cyclophosphamide-treated animals causes an absolute increase in circulating myeloid cells and that these increases are derived from the spleen. The use of recombinant hematopoietic growth factors may permit the administration of more intensive chemotherapy through amelioration of chemically induced leukopenia.
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PMID:Effects of recombinant murine granulocyte-macrophage colony-stimulating factor in cyclophosphamide-treated mice. 201 56

In this study we examined the clinical and laboratory findings of 80 in-patients. There is an important difference between sexes (p greater than 0.05). Comparison of ages showed that 7-30 age is more vulnerable than the older group. We found clinical symptoms of fever, chills, headache, abdominal pain, disturbances in bowel function, nausea, vomiting, anorexia, and lassitude in the first two weeks more frequently when compared with the 3rd, 4th, 5th weeks of illness (p less than 0.001). Where physical finding of rose spots, discordant pulse rate are important in the first two weeks (p less than 0.001). Abdominal discomfort is an important symptom both in the first two and in the last three weeks (% 40.3 and % 36 respectively). Hepatomegaly and splenomegaly, were found more frequently in the last three weeks. According to laboratory findings of anemia, leukopenia, increased erythrocyte sedimentation rate and positive blood and feces cultures there is no important difference between the first two and last three weeks (p greater than 0.05). Increase in polynuclear leucocytes is important for the first two weeks, and increase in lymphocytes is important in the last three weeks (p less than 0.001). Positivity of group agglutination tests is 57%, in the first two weeks and 83% in the last three weeks. This difference is found to be important.
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PMID:[Comparison of symptoms and clinical and laboratory findings in the first and last weeks of typhoid fever]. 208 33

We describe two patients suffering from a prolonged fever, pancytopenia, and splenomegaly. The spleen of both patients was grossly enlarged and the liver was moderately enlarged. The blood pictures were characterized by anemia and leukopenia to a severe degree, with moderate thrombocytopenia and moderate reticulocytosis. The serum transaminases were in the normal range, but the alkaline phosphatase was greatly increased. Splenectomy and wedge liver biopsies were performed on both patients. Histological findings of the spleen were variable. The histopathologic findings of the liver showed infiltration of lymphocytes, plasma cells, monocytes, and neutrophils into the dilated hepatic sinusoids. The sinusoidal infiltrate was more prominent in zones 1 and 2 and less prominent in zone 3 of the acinus. Erythrocyte inclusions that were confirmed by immunohistochemical methods were seen in the cytoplasm of hepatocytes. Electron microscopy revealed a penetration of erythrocytes into the cytoplasm of hepatocytes and caused leakage of cytoplasmic contents into Disse's spaces and sinusoids.
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PMID:Intrahepatocellular erythrocyte inclusions with hepatic sinusoidal infiltrates and splenomegaly. 215 67

Thirty-four new cases of acute promyelocytic leukaemia (M3) were diagnosed at the authors' Centre between 1970 and 1988 (19 males and 15 females) with ages between 5 and 73 years (median age, 32 years). Three cases were of the hypogranular variant or M3-v (8.8%). The clinical picture included: haemorrhagic diathesis (85%), pallor/malaise (82%), fever/infection (41%), hepatomegaly (26%), splenomegaly (12%). Leucopenia of less than 5 x 10(9)/L was present in 23/34 cases, laboratory signs of DIC in 26/31, increased LDH, over 400 U/mL, in 6/31, and abnormal karyotype in 7/15. One of the patients rejected any treatment; two others died of brain haemorrhage before therapy was started, and seven died in the first two weeks of treatment. Of the 31 patients treated, complete remission (CR) was achieved in 21 cases (67.7%). Allogeneic BMT was carried out in two of them, with further relapse and death. Post-remission treatment was given to the remaining 19 patients, and there were 13 relapses. Six patients have been in CR, 5 of them after cessation of therapy, for the last 1.5-11.5 years. Age under 50 years and leucocyte count below 5 x 10(9)/L at diagnosis were favourable prognostic factors according to the univariate statistical analysis performed. The survival plateau of the actuarial curve was reached beyond 2.75 years by 15% of all the patients treated (33 cases), 23% of the patients who achieved CR (21 cases), 31% of the patients under 50 years of age and 5 x 10(9)/L leucocyte count at diagnosis (15 cases) and 36% of these last achieving CR (13 cases).
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PMID:[Acute promyelocytic leukemias: clinico-biological aspects, prognostic factors, therapeutic response, and possibilities of cure in 34 cases (1970-1988)]. 218 63


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