Interleukin-2 after T-cell-depleted allogeneic bone marrow transplantation.
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Adult↗
Biomedical subjects
E Montserrat
Publications and source records attributed to E Montserrat.
Cystic adrenal neuroblastoma: prenatal diagnosis by ultrasonography.
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Adrenal Gland Neoplasms↗
P53 tumor suppressor gene in chronic myelogenous leukemia: a sequential study.
Loss of the p53 gene alleles was investigated in 26 patients with Ph+, BCR/ABL+ chronic myeloid leukemia (CML) by means of the polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP) analysis using the restriction enzyme AccII. In all cases, peripheral blood and/or bone marrow samples were obtained at different times during the chronic phase of the disease and at blast crisis, and in some of them also at the accelerated phase. Of the 12 cases considered informative, 11 evolved into myeloid type blast crisis and one into a lymphoid blast crisis, whereas only two showed an i(17q) chromosome at cytogenetic study. In four of the 12 informative cases, a loss of one p53 gene allele was observed, in all cases coincident with the development of the accelerated phase or blast crisis. One patient with a deleted p53 gene allele, in whom it was possible to analyze the gene structure in the three CML evolutive phases (chronic and accelerated phases and blast crisis), showed loss of the p53 gene allele in both the accelerated and the blastic phase, but not during the chronic phase. On the other hand, one of the two cases with an i(17q) chromosome exhibited one allelic deletion of the p53 gene. Thus, the relatively frequent monoallelic deletion of the p53 gene coincident with the appearance of the blast crisis registered in the present study would support a possible role of the p53 gene alterations in the evolution of CML to its final stages.
Adult↗
Myelomonocytic antigens in B-cell chronic lymphocytic leukemia.
The clinical significance of myelomonocytic (MyMo) antigens in B-cell chronic lymphocytic leukemia (B-CLL) is unclear. We have analyzed the expression of MyMo antigens (CD13, CD14 (LeuM3, My4, Mo2), CD15, CD11b, CD11c, CD33 and CD68) on B-lymphocytes (CD19+) in 105 B-CLL patients and in 35 controls. A double direct staining technique and flow cytometric analysis was performed. The expression of MyMo antigens on the control group did not exceed 4% B-lymphocytes. A MyMo antigen was considered as positive when present in > or = 10% of B-lymphocytes. Among the B-CLL patients, 28 (26.7%) were positive for CD11c, 21 (20.0%) for CD11b, nine (8.6%) for CD15, five (4.8%) for CD13, two (1.9%) for Mo2, and one (1.0%) for My4. No patient was positive for LeuM3, CD33 or CD68. CD11c was more frequently expressed in patients with a short lymphocyte doubling time (< 12 months) (P = 0.05) and CD11b in the group with a higher number of lymphoid areas involved (P = 0.02). No correlation was found between lymphoid morphology and MyMo antigen expression. Fourteen of the 80 patients at risk subsequently progressed to a more advanced stage. Multivariate analysis identified hemoglobin (P = 0.004) and CD11b positivity (P = 0.009) as independent variables for disease progression. Fifteen patients died during evolution. Seven out of the 21 CD11b positive patients and eight of the 84 CD11b negative patients died (LR: P = 0.02, BG: P = 0.05). In the multivariate analysis, only CD11b positivity (> or = 10%) added prognostic value to clinical stages.
Adult↗
Chronic lymphocytic leukemia: present status.
Chronic lymphocytic leukemia (CLL) is the form of leukemia which occurs most frequently in Western countries. Its etiology is unknown, and no relationship with viruses or genes has been demonstrated. Epidemiological data suggest that genetic and ambiental factors might be of some significance. Clinical features of CLL are due to the accumulation of leukemic cells in bone marrow and lymphoid organs as well as the immune disturbances that accompany the disease. The prognosis of patients with CLL varies. Treatment is usually indicated by the risk of the individual patient, which is clearly reflected by the stage of the disease. In the early stage (Binet A, Rai O) it is reasonable to defer therapy until disease progression is observed. By contrast, because their median survival is less than five years, patients with more advanced stages require therapy. For almost 50 years, no major advances in the management of CLL, which has revolved around the use of alkylating agents, have been made. In recent years, the therapeutic approach in patients with CLL has changed as a result of the introduction of combination chemotherapy regimens and, in particular, purine analogues. The latter are already the treatment of choice for patients not responding to standard therapies, and their role as front-line therapy is being investigated. Bone marrow transplants are also being increasingly used. It is to be hoped that in years to come the outcome of patients with CLL will be improved by these advances.
Humans↗
Patients with multiple myeloma requiring long-term dialysis: presenting features, response to therapy, and outcome in a series of 20 cases.
From January 1982 to December 1993, 30 patients with multiple myeloma (MM) required haemodialysis (HD) at our institution. The subgroup of 20 patients who survived more than 2 months on HD is the subject of this study. Four patients were already on HD, due to previous nephropathy, when MM was diagnosed. 13 patients presented with acute renal failure and were on dialysis from the time of diagnosis. The remaining three cases developed renal failure later in the course of the disease. The objective response rate was 40% (8/20). Only two patients could discontinue HD (one had a late partial recovery and one received a kidney graft). Mean hospitalization per year was 19.3 d. The subgroup of patients who survived < 1 year spent a mean of 38.3 d in hospital. Whereas in the subgroup with a survival > 1 year mean hospitalization days was 9.6 (P < 0.001). The median survival was 20 months and six patients survived for > 3 years. In summary, patients with MM and severe renal failure who survive the first 2 months on dialysis have an objective response rate to chemotherapy of 40% and a median survival of almost 2 years, with 30% long-term survivors.
Acute Kidney Injury↗
Presenting features, natural history, and prognostic factors in localized non-Hodgkin's lymphomas: analysis of 117 cases from a single institution.
Clinical features and prognostic factors were analyzed in a series of 117 patients with localized non-Hodgkin's lymphoma (stage I-II). Median age of the patients was 53 years and 52% were men; 22% had a lymphoma of low-grade histology and one-third presented with extranodal involvement. Eighty percent of the patients achieved a complete response (CR); stage of disease and histology were revealed as the most important factors for response. When analysis was restricted to intermediate/high-grade cases, stage showed a predictive value for response. With a median follow-up of 4.5 years, median overall survival was 12.0 years, with 73% and 62.5% of patients being alive at 5 and 10 years, respectively. Main initial parameters significantly related to a shorter survival were intermediate/high-grade histology, stage II, poor performance status, bulky disease, high serum LDH levels, increased ESR, and advanced International Index. In the multivariate analysis, stage, histology and performance status (PS) were statistically significant. Among intermediate/high-grade lymphoma patients, stage and PS provided prognostic value for survival. Twenty-six patients relapsed after CR; median survival after relapse was 2.7 years. Stage (I vs II) was the only predictive variable for relapse in both the whole series and the intermediate/high-grade subset.
Actuarial Analysis↗
Multiple myeloma following essential thrombocythemia.
The association of essential thrombocythemia (ET) and multiple myeloma (MM) is exceedingly rare, with only four such cases reported in the literature until now. In this paper, a patient is reported who developed IgA-lambda MM more than five years after the diagnosis of ET, for which she had received alpha interferon and radioactive phosphor (32P). She died shortly afterwards despite melphalan and prednisone therapy. In four of the five patients in whom the above association has been reported, including the present one, MM appeared some time after ET was initially recognized, with three of them having received alkylating drugs as their treatment for ET. MM was heterogeneous with respect to the M--component type and the clinical course. The possible etiological & pathogenetic link between both these entities is discussed.
Aged↗
[Characteristics of patients with low grade malignant lymphoma and long survival].
PURPOSE: To analyse the initial and evolutive characteristics of the patients with low-grade lymphoma and long survival (> or = 12 years). MATERIAL AND METHODS: The initial data (clinical, laboratory, histologic type, stage) and the clinical course (response to therapy, response duration) of 52 patients with low-grade lymphoma diagnosed before 1980 were analysed. The statistical study was carried out by means of the chi-square test with Yates' correction and Student's t; the actuarial survival was estimated, and the duration of remission was assessed by the Kaplan and Meier method. Curves were compared by the log rank method. RESULTS: The 12-year survival as a whole was 32% (CI: 95%: 18.5%-45.5%). Of the 52 patients, 13 (25%) survived over 12 years. Nine were men and 4 women, with median age of 41 years (range: 22-68 years). Three cases had lymphocytic lymphoma and 10 had follicular lymphoma; 2 were in stage I-II and 11 in stage III-IV. Those patients who would become long-survivors had at diagnosis lesser frequency of spread lymph-node regional involvement (> or = 3 sites): 46% vs 81.8%, p = 0.04, as well as lesser percentage of bone-marrow involvement (38.4% vs 78.1%, p = 0.023). They were also better responders to treatment (CR+PR = 100% vs 71%, p = 0.013) and with longer duration (actuarial median duration of response 8 years vs 1.5 years, p = 0.005). CONCLUSIONS: According to the present analysis, low-grade lymphoma patients who have at diagnosis low tumoral involvement (not too extensive lymph-node involvement, disease-free bone-marrow) and show good response to treatment have a better prognosis and may hope for a longer survival.
Adult↗
[Sepsis by Candida tropicalis in patients with granulocytopenia. A study of 10 cases].
The aim of the present study was to analyze the main clinical and evolutive characteristics of a series of 10 patients diagnosed with sepsis by Candida tropicalis over a 5-year period in a Hematology Unit. The mean age of the 10 patients was 23 years (range 13-66 years) with 6 males and 4 females. Eight patients had acute leukemia, 1 non-Hodgkin's lymphoma and another patient had severe bone marrow aplasia. All the patients presented intense granulocytopenia (< 0.5 x 10(9)/L), had intravenous catheters and were receiving wide spectrum antibiotics as treatment for bacterial infection. The diagnosis of the fungal infection was based on the growth of C. tropicalis in blood cultures together with the evidence of tissue involvement by the fungus. Fever (> 38 degrees C) was the initial symptom of the infection in all the patients, being accompanied by myalgia in 5 cases, pleuritic pain in 2 and septic shock in 1. Violaceous erthymatomous pustules disseminated over the trunk and limbs, the histologic study of which demonstrated the presence of C. tropicalis were observed in 9 patients. Septic metastasis were found in the liver (2 cases), serosae (2 cases), the psoas muscle and the brain (1 case), respectively. Eight patients underwent treatment with amphotericin B which was complemented with 5-fluorocytosin in 6, with death occurring in the remaining 2 patients prior to the start of treatment. Three patients died with active fungal infection (2 by cerebral hemorrhage and 1 by septic shock). In 2 patients the infection evolved to chronic systemic candidiasis and in the remaining 5 patients infection was resolved with hemoperipheral values returning to normal. Sepsis by Candida tropicalis is a severe complication in patients with granulocytopenia, being mainly characterized by fever, cutaneous papulae and, to a lesser extent, muscle pain. Amphotericin B alone, or in combination with 5-fluorocytosin constitute a treatment of choice in this infection, which nonetheless is associated with an undisdainful mortality.
Adolescent↗
Refractory anemia with excess of blasts and isochromosome 12p in a patient with primary mediastinal germ-cell tumor.
Isochromosome(12p), a cytogenetic abnormality characteristic of germ-cell tumors, is exceedingly rare in hematologic disorders. The cytogenetic analysis of a patient diagnosed as refractory anemia with excess of blasts with an associated i(12p), who previously had a teratocarcinoma of the mediastinum, is presented. The cytogenetic analysis was performed at diagnosis of the hematologic malignancy, with all 30 metaphases in the bone marrow culture showing a 47,X, -Y, +8, +i(12)(p10) karyotype. The cytogenetic findings in this patient are compared with those published concerning the association of mediastinal germ-cell tumors and malignant hematologic disorders.
Adolescent↗
PRAD-1/cyclin D1 gene overexpression in chronic lymphoproliferative disorders: a highly specific marker of mantle cell lymphoma.
The t(11;14)(q13;q32) translocation and its molecular counterpart bcl-1 rearrangement are frequently associated with mantle cell lymphomas (MCLs) and only occasionally with other variants of B-cell lymphoid malignancies. This translocation seems to activate the expression of PRAD-1/cyclin D1 gene located downstream from the major breakpoint cluster region of this rearrangement. However, the possible overexpression of this gene in other lymphoproliferative disorders independently of bcl-1 rearrangement is unknown. We have examined the overexpression of PRAD-1 gene in a large series of 142 lymphoproliferative disorders including 20 MCLs by Northern blot analysis. Cytogenetic and/or bcl-1 rearrangement analysis with 2 probes (MTC, p94PS) were performed in 28 cases. Strong PRAD-1 overexpression was observed in 19 of the 20 MCLs including 3 gastrointestinal forms and 4 blastic variants. t(11;14) and/or bcl-1 rearrangement was detected in 6 of the 12 MCLs examined. No correlation was found between the different levels of mRNA expression and the pathologic characteristics of the lymphoma. Among chronic lymphoproliferative disorders other than MCL, only 1 atypical chronic lymphocytic leukemia (CLL) with a t(11;14) translocation and bcl-1 rearrangement and the 2 hairy cell leukemias (HCLs) analyzed showed upregulation of PRAD-1 gene. The expression in the 2 HCLs was lower than in MCL, and no bcl-1 rearrangement was observed. These findings indicate that PRAD-1 overexpression is a highly sensitive and specific molecular marker of MCL but it may also be upregulated in some B-CLLs and in HCL.
Biomarkers, Tumor↗
[Pulmonary cavitation as the initial manifestation of Hodgkin's disease].
Most of the patients with Hodgkin's disease (HD), have lymph nodes in the anterior mediastinum. Nonetheless, it is exceptional for one of these adenopathies to be cavitated at diagnosis. The case of a patient with HD presenting a cavitated anterior mediastinal mass as one of the initial symptoms. The differential diagnosis of localized masses in the anterior mediastinum and cavitated images in conventional thoracic radiology is discussed.
Adult↗
Impact of response to treatment on survival in multiple myeloma: results in a series of 243 patients.
Two hundred and forty-three patients diagnosed with multiple myeloma (MM) in a single institution over a 22-year period and treated with standard chemotherapy were analysed in an attempt to determine the impact of response to therapy on survival. The overall response rate in 229 evaluable patients was 50.1% (34.9% objective response plus 15.2% partial response). Median survivals of patients with objective and partial response were 43.4 and 42.8 months, respectively, versus 19 months for nonresponders. Median survival of 14 patients who achieved a complete remission was 42 months, whereas in 21 rapid responders (< or = 2 months) median survival was 43.3 months. A significant correlation between response and survival was observed with the landmark (P = 0.0169), the Mantel & Byar (P = 0.0001) and the Cox regression model (P < 0.0001) methods. These results indicate that, in responding patients, neither the degree of response nor the response kinetics has a significant influence on survival. However, the response to therapy is associated with a significantly longer survival in MM patients.
Adult↗
Applicability of the International Index for aggressive lymphomas to patients with low-grade lymphoma.
PURPOSE: Variables used to build up the International Index for aggressive lymphomas (age, performance status, stage, extranodal involvement, and lactic dehydrogenase [LDH]) are also important in low-grade lymphoma. To assess the prognostic value of this index in low-grade lymphoma, we have applied it to a series of 125 patients. PATIENTS AND METHODS: One hundred twenty-five patients with low-grade lymphoma who were diagnosed at a single institution over a 20-year period and treated with standard chemotherapy were studied. End points of the study were response to therapy and survival according to the International Index. In addition to the International Index, main initial and evolutive variables were evaluated. Univariate and multivariate methods were used. RESULTS: After applying the International Index, the patients divided into four risk groups: low (36% of cases), low-intermediate (32%), high-intermediate (20.8%), and high (11.2%), with complete response (CR) rates in the four groups being 60%, 35%, 23%, and 21%, respectively. Ten-year overall survival rates for the risk groups were as follows: low, 73.6%; low-intermediate, 45.2%; high-intermediate, 53.5%; and high, 0% (P < .001). When the International Index was included in a multivariate analysis, along with the main initial variables, International Index (P < .001) and sex (male, worse) (P = .038) were the only parameters related to survival. When response to therapy was also included, achievement of CR (P < .0001) and International Index (P < .001) were the most important factors. In patients who achieved a CR, the International Index was the only parameter related to survival (P = .051). The results were the same when the International Index was applied to the subset of 107 patients with follicular lymphoma. CONCLUSION: In this study, the International Index has been found to be an important prognostic tool in low-grade lymphomas. Such an index could be used to predict prognosis not only in aggressive, but also in low-grade lymphomas.
Actuarial Analysis↗
Current approaches to the treatment and management of chronic lymphocytic leukaemia.
Chronic lymphocytic leukaemia (CLL) patients in early clinical stage (Binet A; Rai 0) with nondiffuse bone marrow histopathology and low stable blood lymphocyte levels have long survival rates and should not be treated unless the disease progresses. In contrast, patients with poor prognostic features, such as advanced clinical stage (Binet B, C; Rai III, IV), diffuse bone marrow infiltration or high and rapidly increasing blood lymphocyte levels, have a median survival of less than 5 years and need therapy. Chlorambucil is still the mainstay for CLL treatment. Compared with chlorambucil, combination chemotherapy produces higher remission rates but no increases in survival. New agents such as fludarabine and 2-chlorodeoxyadenosine are already the treatment of choice for patients failing standard therapies, and their role as front-line therapy is being investigated in randomised trials. Whatever the treatment used, however, cure is rarely achieved. Certain situations (e.g. autoimmune cytopenias, hypersplenism) require special treatment approaches (e.g. corticosteroids, splenectomy). Bone marrow transplants, albeit experimental in CLL, warrant investigation in younger patients with poor prognosis.
Antineoplastic Agents↗
Low-grade lymphoma: clinical and prognostic studies in a series of 143 patients from a single institution.
Clinical and prognostic studies were carried out in a series of 143 patients with low-grade (small-lymphocytic, follicular small cleaved cell, follicular mixed small- and large-cell) lymphoma. After treatment with alkylating agents (21.5% cases), combination chemotherapy (73.3%) or other therapies (5.2%), complete response (CR) was obtained in 40.7% of cases and partial response (PR) in 43.7%. The stage of the disease was the most important factor for response. With a median follow-up of 6.5 years, 48.0% (95% Cl: 37.5-58.5) of patients were alive 10 years after diagnosis. Among the initial parameters, advanced stage. B-symptoms, poor performance status, nodal involvement > 3 sites, extranodal involvement > or = 2 sites, WBC count > or = 10 x 10(9)/L, leukemic expression, high serum LDH levels, and bone marrow infiltration were all related to survival; treatment modality, however, had no influence on survival. In the multivariate analysis, stage (p = 0.008) and age (p = 0.053) were the most important prognostic factors. When considering response to therapy, both CR (p < 0.001) and PR (p = 0.003) emerged as the most important predictive variables, with only the absence of B-symptoms retaining its prognostic significance (p = 0.014) among the other parameters. In addition, in CR patients the duration of the response (< or = 1 year vs. > 1 year) was the most significant parameter for survival (p < 0.001). Finally, the initial stage (p = 0.011) and the histologic subtype (those patients with follicular mixed lymphoma relapsing less frequently than the others) (p = 0.052) were the only significant factors for relapse.
Adult↗
Recent progress in chronic lymphocytic leukemia. International Workshop on chronic Lymphocytic Leukemia.
The data we discuss indicate substantial recent progress in understanding and treating CLL. However, despite considerable new information, many of the intriguing issues we posed at previous IWCLL meetings remain unanswered. Prominent among these are the questions of what causes CLL, what is the relation between CLL and normal B-cell development, are T-cell abnormalities a cause of consequence of CLL, why are auto-immune features so prominent and how is CLL best treated? Although these gaps in our knowledge are unfortunate, they give us the opportunity for yet another IWCLL meeting: 1996 in Greece. More to follow.
Antigens, CD↗