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Biomedical subjects

E Montserrat

Publications and source records attributed to E Montserrat.

At least 217 records · Page 12Linked to original sources

Malignant transformation and life expectancy in monoclonal gammopathy of undetermined significance.

The actuarial probability of malignant transformation and the impact on expected survival were analysed in a series of 128 persons diagnosed with monoclonal gammopathy of undetermined significance (MGUS) over a 20-year period. At a median follow-up of 56 months the M-component remains stable in 101 patients (78.9%), 14 patients (10.9%) have died from non-related disorders and 13 (10.2%) have developed malignant transformation of MGUS (multiple myeloma, 10; primary amyloidosis, two; Waldenström's macroglobulinaemia, one). The actuarial probability of malignant transformation at 5 and 10 years was 8.5% and 19.2%, respectively. When different presenting features were analysed for predictive value of the malignant transformation, the IgA type of MGUS was the only variable associated with a higher probability of such an event (P less than 0.025). Although no significant difference was observed between the survival probability of persons with MGUS and that of the control population, the development of malignant transformation was associated with a shorter survival (P less than 0.001).

Adult↗

Sputum sol phase proteins and elastase activity in patients with clinically stable bronchiectasis.

BACKGROUND: Inflammatory and proteolytic activity occurs in sputum from patients with stable purulent bronchiectasis and has been proposed as the main pathogenetic mechanism of the disease. This study was designed to define further the role of inflammation and proteolysis in bronchiectasis. METHODS: Neutrophil elastase activity, sputum concentrations of the serum derived inhibitors alpha 1 antiproteinase and alpha 2 macroglobulin, and the sputum to serum ratios of albumin and C reactive protein concentration were measured in 26 patients with bronchiectasis. RESULTS: Free elastase activity was found in 15 sputum samples. A trend to higher proteolytic and inflammatory activity was found between mucoid and purulent sputum samples, suggesting that inflammatory and proteolytic activities are related to the macroscopic degree of purulence. Purulent sputum had a high sputum to serum ratio of C reactive protein, suggesting local production or active transport of this protein into bronchial secretions. C reactive protein was more sensitive than albumin in detecting a higher degree of inflammation in elastase positive samples. CONCLUSION: The finding of greater concentrations of alpha 2 macroglobulin in purulent and elastase positive samples than in mucopurulent, mucoid and elastase negative sputum samples suggests that this inhibitor may have a role in the proteolysis-antiproteolysis balance in bronchial secretions.

Albumins↗

An unusual case of Castleman's disease restricted to the neck.

An unusual case of Castleman's disease (CD) restricted to the left cervical area is reported. CD is rarely confined to the cervical area; when it occurs, it is usually of the hyaline-vascular subtype and small in size. This case was atypical due to its histology (plasma-cell variant), big size (15 x 11 x 6 cm) and the absence of general symptoms. In spite of surgical excision, the disease recurred 11 months later. However, subsequent surgery followed by chemotherapy resulted in a long-lasting control of the disease.

Adult↗

Polycythaemia vera following non-Hodgkin's lymphoma.

A patient with non-Hodgkin's lymphoma (NHL) who developed polycythaemia vera (PV) is reported. Diffuse large-cell NHL was diagnosed and he was subsequently treated with combination chemotherapy including high dose cyclophosphamide and procarbazine. Four and a half years after chemotherapy splenomegaly developed, coincidently with the appearance of high Hb values, RBC and platelet counts. The screening tests for PV were consistent with this diagnosis, while the search for lymphoma activity was negative. To the best of our knowledge, the present case represents the first well-documented instance of PV following NHL.

Humans↗

Recombinant alpha-2b-interferon may restore natural-killer activity in patients with B-chronic lymphocytic leukemia.

In nine patients with CLL treated with chlorambucil followed by alpha-2b-interferon (alpha 2b-IFN), T, B and natural killer (NK) cells and NK activity were studied before entering the study, after chlorambucil treatment, and after administration of alpha 2b-IFN. When considered as a whole, basal NK activity was lower in CLL patients than in controls (21.0% +/- 10.9 versus 40.2% +/- 17.4, p less than 0.001); however, when considered individually, four out of nine patients had normal NK activity at diagnosis. Chlorambucil did not increase global NK activity (21.7% +/- 7.1), whereas alpha 2b-IFN did so (44.3% +/- 19.1). After alpha 2b-IFN only one of seven patients studied had low NK activity. Previously increased absolute counts of CD2+, CD4+, CD8+, CD16+, CD57+ lymphocytes were reversed with chlorambucil treatment to normal levels, while after this therapy CD11b+ and CD19+ cells decreased without reaching normal values. During alpha 2b-IFN therapy, an increase up to normal levels in the percentage of CD16+ (2.7% +/- 3.4 versus 7.7% +/- 6.5, p = 0.04) and CD57+ (3.0% +/- 3.0 versus 8.1% +/- 6.2, p = 0.020) lymphocytes was observed whereas the absolute number of CD19+ B-cells further decreased (5.2 x 10(9)/l +/- 2.5 vs 3.8 x 10(9)/l +/- 2.3), albeit not significantly.

Adult↗

[Meningeal infiltration in multiple myeloma. Study of a new case and literature review].

A patient with lambda light-chains Bence-Jones multiple myeloma (MM) showed a meningeal myelomatosis during a relapse of his illness. Meningeal infiltration was showed through the detection of plasmatic cells in cerebro spinal fluid, identified morphologic and immunophenotypically, together with hyperproteinemia constituted exclusively by lambda light-chains. Treatment was given, intrathecal (methotrexate and cytosine arabinoside) and systemic (vincristine, adriamycin and dexamethasone) chemotherapy, with disappearance of meningeal infiltration. However the patient died, after three months evolution of MM, tough. Literature on this topic is reviewed.

Adult↗

[Granulocyte alkaline phosphatase activity in the chronic phase and blastic crisis of chronic myeloid leukemia. Sequential study of 43 patients].

PURPOSE: To evaluate in a sequential fashion the activity of the leukocyte alkaline phosphatase (LAP) in the chronic phase and the blastic crisis of chronic myelogenous leukaemia (CML). MATERIAL AND METHODS: This study is comprised of 43 patients diagnosed of CML according to standard criteria. The initial LAP scores were compared with those recorded in the blastic crisis, using cytochemical methods. The statistical analysis was performed with Student's test and chi-square. RESULTS: The LAP activity at diagnosis was low in 40 of the 43 cases (93%), the score being 0 in 20 instances. In the blastic crisis low scores were found in 27 patients (63%), while LAP activity appeared normal or increased in 16 others (37%). When the LAP scores of the two phases of the disease were compared the differences were found statistically significant (p less than 0.0001 and p less than 0.0007, respectively). CONCLUSIONS: These results confirm that the onset of the blastic crisis of CML is often accompanied by an increase of the LAP activity, although this last persists low in the majority of the patients.

Adolescent↗

Presenting features and prognosis of chronic lymphocytic leukemia in younger adults.

We have analyzed 117 younger patients with chronic lymphocytic leukemia (CLL) (mean age, 44.5 years; SD, 4.8; range, 19 to 49; male/female ratio, 2.08) with three main objectives: (1) to see whether these patients have distinctive presenting clinical features; (2) to investigate the impact of the disease on survival; and (3) to analyze whether already well-known prognostic factors are also useful when applied to these patients. As compared with an older age population (greater than or equal to 50 years), there were no major differences in presenting features except for an increased proportion of males (2.08 v 1.21; P less than .025) and a higher hemoglobin level (13.47 +/- 2.70 g/dL v 12.84 +/- 2.77 g/dL; P less than .05) in the younger group. Median survival is 12.3 years (expected median from a control group, 31.2 years). Clinical stages, bone marrow patterns, blood lymphocyte counts, and its doubling time are all useful to separate different risk groups of patients. Whereas patients with favorable prognostic factors have a survival probability of about 80% 14 years after diagnosis, those with poor prognostic features have a median survival of less than 3 years. It is concluded that CLL in younger adults has no major distinctive presenting features and that known prognostic factors are useful to separate different risk groups of patients. These results should be of help in planning therapy for younger persons with CLL.

Adult↗

[T lymphoid blast crisis of Philadelphia chromosome-positive chronic myeloid leukemia: analysis of 3 cases].

BACKGROUND: To investigate the frequency of the T lymphoid phenotype in the blast crisis of chronic myelocytic leukemia (CML) with positive Philadelphia chromosome (Ph) and to evaluate the major clinical and hematologic features of the patients with this phenotype. METHODS: The presence of the TdT enzyme was assessed and specific monoclonal antibodies to the different hemopoietic lines were used to evaluate blast cells in 38 patients with blast crisis during Ph-positive CML. RESULTS: T lymphoid phenotype was found in three of the 38 patients, corresponding in all cases to immature T cells. In one patient, the blast crisis was the presenting feature of CML and in two the localization of the blast crisis was lymphadenopathic. In the two patients who could be followed up a favorable response to chemotherapy regimens including vincristine and prednisone was observed. CONCLUSIONS: Although uncommon, T lymphoid blast crisis in CML should no longer be considered exceptional. This phenotypic finding suggests that, at least in some cases, CML originates in a pluripotential stem cell common to all hemopoietic cells, including T lymphocytes.

Adult↗

Occult cancer in patients with deep venous thrombosis. A systematic approach.

The authors prospectively studied 113 consecutive patients with deep venous thrombosis of the lower extremities to determine the most appropriate workup study for searching for a hidden cancer. After a careful physical examination, the following routine tests were performed: erythrocyte sedimentation rate (ESR), whole blood counts, biochemistry, carcinoembryonic antigen (CEA) levels, chest radiograph, upper gastrointestinal endoscopy, abdominal ultrasound and computed tomography (CT) scan. If a malignant lesion was suspected, further appropriate studies were performed. After discharge, periodic follow-up was performed on all patients in the outpatient clinic. A malignant neoplasm was detected in 12 patients. Of these 12 patients, six were asymptomatic with the exception of experiencing thrombophlebitis. Cancer was found more commonly in patients with idiopathic deep vein thrombosis (DVT) (7 of 31 versus 5 of 82 patients with secondary DVT; P = 0.012), and in those patients with abnormal lactic dehydrogenase (LDH) levels (6 of 23 versus 6 of 90; P = 0.007). Abnormal CEA levels allowed diagnosis of two cases of colonic cancer (on colonoscopy). Both ultrasound and CT scan of the abdomen showed two cases of urinary bladder carcinoma at a very early stage. Furthermore, two cases of adenomatous polyps in colon were found, a condition considered by most authors to be a colorectal cancer precursor. In addition, there were five patients with large benign pelvic tumors, and two patients with absent inferior vena cava. The most striking finding was that some cases of cancer were at a very early stage. It was concluded that blood cell counts, LDH, CEA, chest radiograph, and abdominal ultrasonography (or CT scan) should be routinely performed on all patients with deep venous thrombosis (particularly those with idiopathic DVT). Malignancy would not have been recognized in some patients if these tests had not been performed.

Adult↗

Treatment of early stage-B chronic lymphocytic leukemia with alpha-2b interferon after chlorambucil reduction of the tumoral mass.

Eleven patients with early CLL (two in Rai's stage 0, seven in stage I, and two in stage II) received aIFN (3 MU subcutaneously three times a week for a median of 8 months; range, 4-12) after their tumor mass had been reduced with intermittent chlorambucil. Following chlorambucil/aIFN administration, a significant reduction in blood lymphocyte counts (from 25.1 +/- 12.0 x 10(9)/l to 6.3 +/- 5.32 x 10(9)/l; p less than 0.001) and in CD 19-positive cells (from 21.0 +/- 12.0 x 10(9)/l to 3.8 +/- 2.3 x 10(9)/l; p less than 0.001) was observed. Three of seven patients with stage-I and one of two with stage-II disease moved to stage 0, and a complete response (CR) was observed in two patients with stage-I at diagnosis. Overall, there were eight patients who, after treatment, had either a CR (2 cases) or stage-0 disease (6 cases), which compares favorably with two patients with stage-0 disease before entering the study (p = 0.015). In five patients (including the two who achieved a CR) aIFN further improved the disease status achieved with chlorambucil.

Adult↗

alpha Interferon in chronic lymphocytic leukaemia.

The role of alpha interferon in patients with chronic lymphocytic leukaemia (CLL) has yet to be well established. In studies carried out to date, a significantly higher response rate has been observed in previously untreated patients compared to those who have received prior chemotherapy. Patients with early-stage CLL also respond better than patients with advanced disease. Responses to alpha interferon are transient and complete responses are rare. It is not yet known whether alpha interferon can induce clonal remission, and response is usually measured in terms of the reduction in peripheral blood lymphocyte levels. In one study, a normalization of immunoglobulin levels was observed, and in another there was an increase in the absolute number of granulocytes. Further studies are needed to investigate the role of combined therapy with alpha interferon and cytotoxic agents or other cytokines, and to assess the ability of alpha interferon to prolong response duration after remission induction with chemotherapy. Toxicity is tolerable when alpha interferon is given in a low dose (e.g., 2 million units (MU)/m2 three times a week) and low doses have been shown to be as effective as high doses in CLL patients.

Clinical Trials as Topic↗

Xanthogranulomatous pyelonephritis and systemic amyloidosis: report of 2 new cases and the natural history of this association.

The association of xanthogranulomatous pyelonephritis and systemic amyloidosis is extremely rare. To our knowledge, despite innumerable cases of xanthogranulomatous pyelonephritis reported in the literature, this association has been described on only 2 occasions. Clinical, analytical and radiographic findings can be highly suggestive of the diagnosis. We report on 2 patients with xanthogranulomatous pyelonephritis, systemic amyloidosis and the nephrotic syndrome: 1 died 4 1/2 years after diagnosis and 1 was stable with good general health 3 years after diagnosis. The clinical aspects as well as the treatment given to both patients are discussed. We describe the natural history of an association that due to its rarity is not currently well known.

Adult↗