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J M Ribera

Publications and source records attributed to J M Ribera.

At least 73 records · Page 4Linked to original sources

[Primary gastrointestinal lymphomas: response to eradicative therapy and prognostic factors in 52 patients].

BACKGROUND: To analyze the response to eradicative therapy and prognostic factors in 52 patients with primary gastrointestinal lymphoma (PGIL) diagnosed at a single institution in a 13 year period. PATIENTS AND METHODS: The main clinical, biological and evolutive data were recorded. Pathologic diagnosis of PGIL was made according to the Working Formulation. Clinical stage was determined by the Ann Arbor system modified by Mushoff. The results of therapy as well as the influence of such characteristics on complete remission (CR), disease-free survival (DFS) and overall survival (OS) were studied. RESULTS: Mean age of the series was 53 years (SD 15). Thirty patients were males. HIV infection preceded PGIL diagnosis in 10 cases. Seventeen had bad performance status (ECOG 2-4) and 30 B symptoms. The PGIL localization was gastric in 31 cases and 29 had a low grade malignant lymphoma. B phenotype was demonstrated in 98% and 22 patients presented advanced stages (IIE2-IV). Treatment was radical surgery followed by intensive chemotherapy in 32 cases, intensive chemotherapy alone in 17, and surgical resection in 3. CR was obtained in 34 patients and 6 of them relapsed. The projected DFS from CR at 9 years was 72% and OS was 26%. CR and survival were not influenced by PGIL localization and treatment type. The main unfavourable prognostic factors were advanced stage (CR and OS), B symptoms (DFS and OS) and advanced ECOG score (CR, DFS and OS). Previous HIV infection had an independent prognostic influence on both CR and OS. CONCLUSIONS: In patients with PGIL, the achievement of CR, DFS and survival have been independent of the type of eradicative treatment used. Performance status, B symptoms and clinical stage have been the main prognostic factors. HIV infection carried an independent prognostic significance.

Adult↗

Peripheral T-cell lymphomas: initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. Classification.

BACKGROUND: Peripheral T-cell lymphomas (PTCL) account for about 10% of all lymphomas in Western countries. The aim of the present study is to analyze the initial characteristics and prognostic factors in a large series of PTCL patients. PATIENTS AND METHODS: 174 patients (105 male/69 female; median age 61 years) were diagnosed with PTCL according to the R.E.A.L. Classification in nine Spanish institutions between 1985 and 1996. Cutaneous lymphomas and T-cell chronic lymphocytic/prolymphocytic leukemia were excluded from the study. Univariate and multivariate analyses were used to assess the prognostic value of the main initial variables. RESULTS: The distribution according to histology subgroup was: PTCL unspecified, 95 cases (54.4%); anaplastic large-cell Ki-l-positive (ALCL), 30 cases (17%); angioimmunoblastic T cell, 22 cases (13%); angiocentric, 14 cases (8%); intestinal T cell, 12 cases (7%), and hepatosplenic gamma delta T cell, one case (0.6%). As compared to the other types, ALCL presented more frequently in ambulatory performance status, without extranodal involvement, in early stage, normal serum beta 2-microglobulin (B2M) level and low-risk international prognostic index (IPI). Most patients were treated with adriamycin-containing regimens. The overall CR rate was 49% (69% for ALCL vs. 45% for other PTCL; P < 0.02). The risk of relapse was 48% at four years. Median survival of the series was 22 months (65 months for ALCL vs. 20 months for other PTCL; P = 0.03), with a four-year probability of survival of 38% (95% confidence intervals (95% CI): 28-48). In the univariate analysis, in addition to the histology, older age, poor performance status, presence of B-symptoms, extranodal involvement, bone marrow infiltration, advanced Ann Arbor stage, high serum LDH, high serum B2M, and intermediate- or high-risk IPI were related to poor survival. In the multivariate analysis the histologic subgroup (ALCL vs. other PTCL) (P = 0.02; response rate (RR): 4.3), the presence of B-symptoms (P = 0.02, RR: 2.2), and the IPI (low vs. high) (P = 0.04, RR: 2) maintained independent predictive value. When the analysis was restricted to the unspecified subtype, only IPI had independent prognostic value (P = 0.003; RR: 3.5). CONCLUSIONS: PTCL have adverse prognostic features at diagnosis, respond poorly to therapy and have short survival, with no sustained remission. ALCL constitutes a subgroup which responds better to therapy and has a longer survival.

Adolescent↗

Acute myeloblastic leukemia with minimal myeloid differentiation: phenotypical and ultrastructural characteristics.

AML-M0 is an infrequent form of acute myeloblastic leukemia characterized by negative reaction with myeloperoxidase (MPO), Sudan Black and lymphoid antigens and positivity for CD13 or CD33. In the present study we describe the immunophenotypical and ultrastructural characteristics of a group of AML-M0 in adult patients. Nine out 218 AML leukemias (4.1%) fulfilled the AML-M0 criteria. CD13 or CD33 were positive in eight out nine cases, with two or more positive myeloid antigens being present in 82% of the cases. Immunological MPO was positive in 57% of the cases and CD68 in 33%. In no case megakaryocytic and erythroid markers present. Four cases (44%) expressed CD7 and TdT but only two coexpressed both antigens. In none of the cases was CD3 or CD22 cytoplasmic expression found. Ultrastructurally, a low number of granules was seen in all cases whereas ferritin particles or rhopheocytosis were not observed. Ultrastructural MPO was positive in one out of five cases and platelet peroxidase (PPO) was negative in the four cases studied. Two out of six cases showed karyotypic abnormalities (hypotetraploidy and a complex karyotype, respectively). In two out three cases a rearranged pattern for JH gene was observed. TCR (Cbeta and Jgamma) rearrangements were not detected in any case. AML-M0 is an infrequent form of acute myeloblastic leukemia. A large panel of myeloid monoclonal antibodies (MoAb) and the study of the cytoplasmic expression of myeloid antigens is necessary to diagnose this form of leukemia. AML-M0 usually coexpress lymphoid markers. Ultrastructural studies may be of help to discard an immature erythroid proliferation.

Adult↗

Essential monoclonal gammopathy with an IgM paraprotein that is a cryoglobulin with cold agglutinin and EDTA-dependent platelet antibody properties.

A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. Serum was studied to determine the relationship of the cryoglobulin with the cold agglutinin. The cryoglobulin and cold agglutinin were found to be the same paraprotein. Moreover, with absorption and elution techniques the reactivity of the autoantibody with both erythrocytes and platelets was demonstrated. Reports of cryoprecipitable cold agglutinins are rare and therefore this case is exceptional given that the IgM kappa paraprotein was found to be a cold agglutinin which was also reactive with platelets.

Aged↗

Late intensification chemotherapy has not improved the results of intensive chemotherapy in adult acute lymphoblastic leukemia. Results of a prospective multicenter randomized trial (PETHEMA ALL-89). Spanish Society of Hematology.

BACKGROUND AND OBJECTIVE: Intensive induction and post-remission therapies have improved the prognosis in adult acute lymphoblastic leukemia (ALL). However, different from children, the impact of late intensification therapy in the overall results of treatment has not been consistently evaluated. The objective of this study was to analyze the results of a multicenter prospective protocol, PETHEMA ALL-89, in which, after intensive induction and consolidation therapy, randomization to receive delayed intensification treatment was performed. DESIGN AND METHODS: One hundred and eight adults (age > or = 15 years) diagnosed with ALL (ALL L3 excluded) in 22 Spanish hospitals from 1989 to 1994 were treated with a five-drug induction therapy, followed by four cycles of early post-remission treatment during four months, and maintenance therapy for two years. Patients in remission at the end of the first year were randomized to receive one six-week cycle of late intensification therapy. Uni- and multivariate analyses of early response to treatment, complete remission (CR), leukemia-free survival (LFS) and overall survival (OS) were performed. RESULTS: The median (range) age of the series was 28 (15-74) years and leukocyte count 26 x 10(9)/L (1-600). ALL L1/L2 was present in 38/70 patients, early pre-B in 13, common in 53, pre-B in 12 and T in 30 cases. The CR rate was 86%, and refractory disease 9%. Median LFS was 34 months, with a 5-yr probability of 41% (95% CI, 29-53), whereas median OS was 51 months and 5-year probability 47% (34-59%). There were no differences in either LFS and OS between patients who did or did not receive delayed intensification therapy. Prognostic factors for CR attainment were advanced age and slow response to therapy. These two features were, in addition to high leukocyte counts, the parameters with negative influence in both LFS and OS. INTERPRETATION AND CONCLUSIONS: The results of PETHEMA ALL-89 are similar to those referred in other chemotherapy-based protocols in adult ALL. Delayed intensification has not improved the length of remission and survival. Efforts to improve the prognosis of adult ALL patients must be mainly focused in early intensification treatment.

Adolescent↗

International prognostic index is the best prognostic factor for survival in patients with AIDS-related non-Hodgkin's lymphoma treated with CHOP. A multivariate study of 46 patients.

BACKGROUND AND OBJECTIVE: The management of non-Hodgkin's lymphomas (NHL) in AIDS is difficult because of the poor bone marrow reserve and immunosuppression of these patients. Combination chemotherapy is the treatment of choice in the subset of patients with good performance status and mild immunosuppression. Several combination chemotherapy regimens have been used in these patients but the results have been poor. We have studied the clinical and biological features, response to treatment, outcome and prognostic factors of 46 patients with NHL and HIV infection, diagnosed in a single institution between January 1988 and June 1997. DESIGN AND METHODS: Forty-six patients with NHL and HIV infection were treated with CHOP. Patients with previously treated systemic NHL, primary CNS NHL, performance status > 2 and active AIDS-defining opportunistic or neoplastic diseases were excluded. The parameters evaluated were: age, sex, risk activity, basic hematologic and biochemical parameters, CD4 lymphocyte count, B symptoms, stage (Ann Arbor), histologic subtype (REAL classification), International Prognostic Index (IPI), response to treatment, relative dose intensity (RDI), relapse free survival (RFS) and overall survival (OS). Uni- and multivariate analyses of prognostic factors were performed. RESULTS: Median age was 35 years and 40 patients were male. CD4 lymphocyte count was lower than 0.1 x 10(9)/L in 18 out of 38 cases, hypoalbuminemia was registered in 24 (52%), serum LDH was higher than 400 U/L in 20 (43%) and beta 2-microglobulin was higher than the normal range in 9 out of 20 patients (45%). Complete response was achieved in 18 patients (40%). Twenty-six patients received G-CSF after chemotherapy. Grade 4 neutropenia and fever were significantly more frequent in patients who did not receive G-CSF. Serum LDH > 400 U/L and hypoalbuminemia were the only parameters associated with a lower probability to achieve complete response (p = 0.015 and p = 0.025, respectively). The median RFS was 26 (6-47) months and no variable was found to have statistically significant influence on it. The median OS was 9.2 (4.5-14) months, and IPI score 1 and ESR < 60 mm/h were the only parameters identified as good prognostic factors in the multivariate analysis (p = 0.03 and 0.049, respectively). INTERPRETATION AND CONCLUSIONS: In spite of patient selection, the response to CHOP treatment in patients with NHL and HIV infection remains poor. Episodes of neutropenic fever are less frequent when G-CSF is administered after CHOP. The IPI score 1 is the most important favorable prognostic factor for survival.

Adult↗

Phenotypic changes in neutrophil granulocytes after G-CSF administration in patients with acute lymphoblastic leukemia under chemotherapy.

Phenotypic changes in neutrophil granulocytes (NG) after G-CSF have been scarcely studied. Using flow cytometry, we analyzed the changes of CD11b, CD14, CD33, CD71, HLA-DR, CD10, CD16 and CD15 on NG after G-CSF treatment in 6 patients with ALL receiving intensification chemotherapy and in 10 control subjects. After G-CSF we found: expression of HLA-DR, a higher expression of CD11b, CD71 and CD14, a decrease in CD10 positivity, and fluoresence Intesity in CD15 and CD16. After administration of G-CSF, the NG of patients with ALL express an immature phenotype as well as markers of proliferation.

Adult↗

Prophylactic platelet transfusion threshold during therapy for adult acute myeloid leukemia: 10,000/microL versus 20,000/microL.

BACKGROUND AND OBJECTIVE: The threshold for prophylactic platelet transfusions has been classically established at 20,000/microL. In 48 patients with de novo acute myeloblastic leukemia (AML) we analyzed the effect of reducing the threshold for prophylactic platelet transfusion from 20,000/microL (group A) to 10,000/microL (group B) after induction and consolidation chemotherapy. DESIGN AND METHODS: Forty-eight adult patients with de novo AML diagnosed in a single institution in a nine year period were enrolled in the study. Between January 1989 and December 1993 the patients received prophylactic platelet transfusions when their platelet count was below 20,000/microL (group A), and from January 1994 to March 1998 prophylactic platelet transfusions were indicated below 10,000/microL or between 10,000/microL and 20,000/microL if there was any consumption factor. RESULTS: The mean number (SD) of platelet transfusions during induction was 8.4 (5.3) in group A and 8.5 (5.5) in group B; and during consolidation 4.7 (3.4) in group A and 4.6 (3.8) in group B (p = n.s.). Excluding the cases with consumption factors from the analysis, group B patients required 34% fewer transfusions during induction and 15.5% fewer during consolidation (p = 0.04). There were no differences between groups regarding major bleeding episodes. INTERPRETATION AND CONCLUSIONS: Our data show that the threshold for prophylactic platelet transfusion can be safely set at 10,000 microL during induction and consolidation chemotherapy for adult patients with de novo AML.

Acute Disease↗

[Survey of residents on their satisfaction after obtaining a hospital post].

BACKGROUND: To evaluate the degree of satisfaction of the resident physicians in an university hospital by the election of the speciality and the hospital, after the takeover of the workplace and in the course of their period of residence. SUBJECTS AND METHODS: An anonymous questionnaire was distributed to the 164 residents of the hospital to know the concordance between the initial expectations of the residents and the degree of current satisfaction, about the specialty and the chosen hospital. RESULTS: 149 out of 164 residents (90.8%) answered the survey. In what is referring to the elected speciality, 57% (85 residents) expressed to be satisfied or very satisfied by their election; 22% (33 residents) felt indifferent, and 21% (31 residents) were unsatisfied or very unsatisfied. In what concerns to the chosen hospital, 43% (64 residents) were satisfied or very satisfied; 29% (43 residents) indifferent, and 28% (42 residents) were unsatisfied or very unsatisfied. With respect to the degree of satisfaction by the exercise of the profession at the moment of accomplishing the survey, 98% (143/149) of the residents answered, and of these, 30% (43 residents) expressed to be satisfied or very satisfied; 28% (40 residents), indifferent, and 42% (60 residents), unsatisfied or very unsatisfied. In the degree of satisfaction influenced in a statistically significant way the group of speciality chosen (medical, surgical or central services) after the examination and the moment of accomplishing the survey; the number of order of the residents examination; to have or not to have a speciality wished before of the residents examination, and the year of residence underway. CONCLUSIONS: The degree of satisfaction of the resident physicians by the election of the speciality and of the hospital is relatively low. This fact outlines the suitability of the election system of the specialty.

Adult↗

[Spinal cord compression in non-Hodgkin's lymphoma. A study of 10 patients].

Spinal cord compression is an infrequent event in patients with non-Hodgkin's lymphomas (NHL) and early diagnosis and therapy are required. The main clinical and histologic characteristics as well as the response to therapy in 10 NHL patients with spinal cord compression diagnosed at a single center in a 7-year period are referred. The main initial clinical manifestations were pain in dorsal or lumbar regions (5 cases) and paraparesia or paraplegia (5 patients). Infection by human immunodeficiency virus (HIV) was present in four cases. In 8 patients spinal cord compression was the initial manifestation of NHL. Pathologic diagnosis showed intermediate or high-grade lymphoma in 8 out of 10 cases and immunologic phenotype was B-cell in all cases. Laminectomy followed by radiation therapy and chemotherapy was performed in 4 cases, radiotherapy and chemotherapy in two, isolated chemotherapy in 3 and radiation therapy was administered to the remaining case. Complete response was observed in 4 cases and 6 cases were resistant to treatment. No patient with HIV infection responded to treatment. Six patients had died, median survival time for the whole series being 4 months, and the actuarial probability of survival was 40% at the first year.

Adult↗

[Hemopoietic progenitor cell transplantation: 20 years' experience at the Hematology School "Farreras-Valentí"/Clinical Hospital of Barcelona].

BACKGROUND: Hemopoietic progenitor cell transplantation (HPCT) is acquiring an increasing role in the therapy for a variety of disorders. In this study, main characteristics and results of HPCT along 20 years are analyzed from the experience of Postgraduate School of Hematology "Farreras-Valentí" at the Hospital Clínic in Barcelona. PATIENTS AND METHODS: Six-hundred ninety-five patients transplanted between June 1976 and January 1996 were analyzed. Median age (range) were 33 (4-63) years. The following aspects were considered: donor type, source or progenitor cells, type of disease and disease-stage at transplantation, transplant related mortality and survival. RESULTS: A total of 714 HPCT were performed (448 allogeneic, 13 isogeneic, 253 autogeneic). Allogeneic HPCT were from an HLA-identical sibling in 408 cases, from other familial donors in 10, and from non-familial donors in 30. Most HPCT from non-familial donors (93%) were performed during the last five years of the study (1991-1995). The source of hemopoietic progenitor cells was bone marrow in 625 instances (88%), peripheral blood in 88 (12%), and fetal liver in one. During more than 15 years, the only source of progenitors was the bone marrow; in contrast, in the last 3 years (1993-1995) transplants using peripheral blood were predominant. Main indications for HPCT were the following: acute leukemias (n = 387) (54%), chronic leukemias (n = 134) (19%), severe aplastic anemia (n = 58) (8%), lymphomas (n = 80) (11%), multiple myeloma (n = 39) (5%) and myelodysplastic syndromes (n = 14) (2%). In patients with hematological malignancies (n = 656), HPCT was performed in first complete remission or in first chronic phase in 321 instances (49%), in subsequent remissions in 144 (22%), and in more advanced stages in the remaining 191 (29%). In the more recent years, a progressive decrease in the number of HPCT for acute leukemia or aplastic anemia was observed, contrasting with an increase in transplants for lymphoma, multiple myeloma and myelodysplastic syndromes. Of note, a significant decrease in transplant related mortality was evident along the years, both after autogeneic HPCT (21% during 1985-1992 and 6% thereafter) (p = 0.001) and after allogeneic transplantation (54%, 44%, and 20% during the periods 1976-1984, 1985-1992 and 1993-1995, respectively) (p = 0.004). The fact translated into an increase in the actuarial probability of survival after allogeneic HPCT (25%, 33% and 58% in the three mentioned periods, respectively) (p = 0.0003), and after autogeneic HPCT (33% in the interim 1985-1992, and 55% in the period 1993-1995) (p = 0.001). CONCLUSIONS: During the last 20 years, HPCT has significantly evolved in aspects such as type of donor, source of progenitor cells and indications. Remarkably, a progressive decrease in transplant related mortality has been observed translating into a improvement in survival after the procedure.

Actuarial Analysis↗

[Acute myelomonocytic leukemia with eosinophilia and inversion of chromosome 16. Study of 6 cases].

The purpose of this study has been to refer the main clinico-biologic characteristics, the evolution and the response to therapy in 6 patients with acute myelomonocytic leukemia with eosinophilia and inversion of chromosome 16 (AML4Eo inv[16]) belonging to a series of 92 patients with acute myeloblastic leukemia diagnosed in a single hospital between 1987 and 1995. The main clinical manifestations were anemic syndrome and hemorrhage. Anemia and thrombocytopenia were present in all cases, high white blood cell count in 4, monocytosis in 5 and eosinophilia in one. Bone marrow aspirate showed myeloid and monocytic blast infiltration (43-62%), eosinophilia (5-19%) and atypical monocytic precursors (6-18%). Induction therapy consisted in one or two cycles of daunorubicin (or idarubicin), cytosine arabinoside and etoposide, followed by two cycles of consolidation treatment, the first with mitoxanthrone and cytosine arabinoside and the second with amsacrine and cytosine arabinoside. One patient died in the induction phase, while complete remission was obtained in the remaining 5. One patient died during the consolidation therapy. Allogeneic bone marrow transplant (BMT) was performed to one patient and autologous BMT to another. The first patient remains in complete remission (CR) at 68 months from diagnosis, and the second relapsed 12 months after BMT. Another patient relapsed at 13 months from diagnosis and the remaining persists in CR 13 months from diagnosis. Actuarial probabilities of CR duration and survival were 50% at 5 years. The clinico-biologic characteristics and the response to therapy of patients with AML4Eo inv(16) are similar to those referred in other series. There is a high probability of CR attainment and, probably, the relapse rate is lower than that of other subtypes of AML.

Adolescent↗

CYP2D6, NAT2 and CYP2E1 genetic polymorphisms in nonagenarians.

BACKGROUND: enzymatic polymorphisms affecting the metabolic disposition of xenobiotics may modulate the rate of activation or deactivation of carcinogens and other toxic environmental chemicals. Hence, these polymorphisms may influence the risk of suffering some types of cancer and other degenerative diseases that are incompatible with extreme longevity. AIMS: to establish the distribution of three well known enzymatic polymorphisms that affect the CYP2D6, NAT-2 and CYP2E1 genes and the activity of their enzymatic gene products, involved in the disposition of many xenobiotics, in a group of nonagenarians and in much younger controls. PATIENTS: the three genotypes were determined in 41 nonagenarians (10 males, mean age 92.2 years, range 90-98) free of known malignancies or neurodegenerative diseases. The control groups comprised 217 healthy volunteers (128 males, mean age 36.3 years; SD, 12.7) for the CYP2D6 and NAT2 genotypes and 137 (116 males, mean age 32 years; SD, 18.8) for the CYP2E1 genotype. METHODS: after extraction of DNA from white blood cells, polymerase chain reaction and restriction fragment polymorphism methods were used to identify the allelic variants of the three genotypes. RESULTS: we found no qualitative or quantitative difference in the mutations underlying the three genetic polymorphisms studied, nor in the expected enzymatic phenotypes. Instead, a close parallelism exists between advanced age and younger groups. CONCLUSION: longevity does not seem to be related to any special configuration of these three polymorphic traits. Comparisons with younger controls may be adequate when studying the distribution of these polymorphisms in diseases affecting old people. No genetically determined differences in the activation of drugs metabolized by these enzymes are to be expected in very old people.

Aged↗