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Biomedical subjects

A Delmer

Publications and source records attributed to A Delmer.

At least 55 records · Page 3Linked to original sources

Multiple lymphomatous polyposis of the gastrointestinal tract: prospective clinicopathologic study of 31 cases. Groupe D'étude des Lymphomes Digestifs.

BACKGROUND & AIMS: Multiple lymphomatous polyposis (MLP) is an uncommon type of gastrointestinal lymphoma. The aim of this study was to analyze the clinicopathologic features and outcome of a large series of patients. METHODS: From 1984 to 1995, 31 patients were enrolled prospectively. Exhaustive staging and immunohistochemical analysis were performed. The first 10 patients were treated with cyclophosphamide-vincristine-prednisolone (COP) and the subsequent 21 with doxorubicin-teniposide-cyclophosphamide-prednisolone (AVmCP). Eleven patients were treated with high-dose radiochemotherapy and stem cell autotransplantation because of partial response or relapse. RESULTS: Advanced age and multiple polyps involving several gastrointestinal segments characterized initial clinical presentation. The typical tumor cell phenotype (pan-B+, CD5+, and CD10-) along with Bcl-1 rearrangement indicated that MLP is the gastrointestinal counterpart of mantle cell lymphoma. After COP treatment, only 3 patients achieved partial remissions, whereas 80% of patients after AVmCP treatment achieved complete or good partial remission. High-dose radiochemotherapy changed partial into complete remission in 80% of cases. None of the initial 10 patients survived for 3 years, but the expected 5-year survival rate was 59% in patients receiving the anthracycline-containing regimen (P < 0.0001). CONCLUSIONS: MLP is a distinct entity among gastrointestinal lymphomas. An anthracycline-containing multidrug regimen and high-dose radiochemotherapy supported by autotransplantation seem to improve its poor prognosis.

Adult↗

Treatment of hairy cell leukaemia (HCL) with 2-chlorodeoxyadenosine (2-CdA): identification of parameters predictive of adverse effects.

2-chlorodeoxyadenosine (2-CdA) induces high complete remission (CR) rates in hairy cell leukaemia (HCL), but is associated with serious toxicities. Therefore we reviewed our experience with 2-CdA in 16 HCL patients, with special attention to adverse effects. One-third of patients presented severe neutropenic infections and/or required prolonged blood support. Patients with low tumour mass and moderate cytopenias were more likely to achieve CR, whereas those with high tumour burden and severe bone marrow impairment were at increased risk of severe infection and blood product requirements. All these unfavourable parameters may be corrected by short-term alpha-interferon (IFN) therapy. Therefore we suggest that patients with unfavourable presenting features might benefit from IFN therapy before 2-CdA.

Antineoplastic Agents↗

Cefepime/amikacin versus ceftazidime/amikacin as empirical therapy for febrile episodes in neutropenic patients: a comparative study. The French Cefepime Study Group.

We conducted a randomized multicenter study to compare the efficacy and safety of two antibiotic regimens (cefepime [2 g b.i.d.] plus amikacin or ceftazidime [2 g t.i.d.] plus amikacin) as first-line therapy for fever in patients with hematologic malignancies and neutropenia. A total of 353 patients were randomized according to a 2:1 (cefepime:ceftazidime) ratio. Two hundred-twelve patients in the cefepime group and 107 in the ceftazidime group (90% of all patients) were evaluable for efficacy. The polymorphonuclear neutrophil count was < 100/mm3 on enrollment for 70% of the patients. The mean duration of neutropenia was 26 days. The efficacy in both study arms was comparable, although a trend in favor of cefepime was seen in terms of therapeutic success (response rate, 27% vs. 21% for the ceftazidime group). The overall response rate after glycopeptides were added to the regimens was 60% for the cefepime group and 51% for the ceftazidime group; the bacterial eradication rates were 81% vs. 76%, respectively, and the rates of new bacterial infections were 14% vs. 18%, respectively. We conclude that the combination cefepime/amikacin is at least as effective as the reference regimen of ceftazidime/amikacin in this setting.

Amikacin↗

[Chronic myelomonocytic leukemia in adults].

Chronic myelomonocytic leukemia (CMML) is characterized by the association of myelodysplastic features and proliferation of both granulocytic and monocytic series. This disease frequently indolent, may however demonstrate progressive course and/or transform to acute leukemia. Nosologic and diagnostic problems raised by this malignant hematological pathology, its clinical and biological presentations, and current treatments are reported in this review.

Adult↗

Treatment of agnogenic myeloid metaplasia with danazol: a report of four cases.

Peripheral cytopenias are common in patients with agnogenic myeloid metaplasia (AMM). They are an important cause of morbidity and mortality, and their treatment is difficult. We report on 4 patients with AMM and severe cytopenia treated with danazol (400-600 mg/ day). Three of them became independent of red blood cell (RBC) transfusion, while the other had a slight reduction in RBC requirement. In addition, correction of thrombocytopenia and disappearance of splenomegaly were observed in 1 and 2 patients, respectively. No side effects were observed. In our experience, danazol appears effective and safe in the subset of patients with AMM whose disease is mainly characterized by bone-marrow failure. These data warrant further studies to evaluate this treatment and explore its mechanism of action.

Aged↗

Scleritis and mucosal-associated lymphoid tissue lymphoma: a new masquerade syndrome.

PURPOSE: To present a new masquerade syndrome showing features of mucosal-associated lymphoid tissue (MALT) lymphoma associated with choroidal white dots and scleritis. Differentials including systemic lymphoma, central nervous system lymphoma, and etiologies of white-dot syndromes and scleritis are discussed. PATIENTS AND METHODS: A 42-year-old man who had decreased vision and ocular redness of his right eye for 4 years had a biopsy-proven diffuse anterior and posterior scleritis associated with intense circumferential perilimbal chemosis and ipsilateral yellow-white choroidal dots. A new conjunctival biopsy was performed because of unresponsiveness to high-dose systemic steroid and cyclophosphamide therapy. Immunostains for lymphocyte markers were preformed. RESULTS: A morphologically and immunohistochemically typical, monotypical mu-kappa immunoglobulin light chain secreting B-cell MALT-lymphoma was diagnosed. Eighteen months after completion of radiotherapy, the patient recovered completely, except for the choroidal dots, which remained unchanged. CONCLUSION: When scleritis, even histologically proven, fails to respond to immunosuppressive therapy, a new biopsy is mandatory to rule out a misdiagnosed MALT lymphoma.

Adult↗

[Diffuse normolipidemic plane xanthomatosis and monoclonal dysglobulinemia. A case with anti-lipoprotein activity of monoclonal immunoglobulin and hypocomplementemia].

INTRODUCTION: Diffuse normolipaemic plane xanthomatosis associated with monoclonal dysglobulinaemia has been recognized as a clinical entity for more than 30 years. Antilipoprotein activity has been reported in certain cases with monoclonal immunoglobulinaemia or hypocomplementaemia. CASE REPORT: A 64-year-old woman developed simultaneously diffuse normolipaemic plane xanthomatosis and monoclonal IgG kappa dysglobulinaemia. This paraprotein was initially associated with monoclonal gammapathy of undetermined signification (called "benign") before evolving into a myeloma. This aggravation was associated with an expansion of the skin lesions. Immunological exploration demonstrated the presence of IgG-lipoprotein complexes in the plasma and hypocomplementaemia suggesting incomplete activation of the classical pathway (low CH50 and fraction C4). DISCUSSION: According to the literature, the most probable hypothesis for the pathogenesis of the xanthomatosis-dysglobulinaemia association is a specific interaction between the monoclonal immunoglobulin and lipoprotein metabolism. In normolipaemic forms, the immunoglobulin-lipoprotein complexes are recognized by "waste-receptors" and accumulate in the dermal macrophages while the mechanism of lipoprotein captation in the other types of cells remains normal, explaining the absence of hyperlipidaemia.

Complement System Proteins↗

[Pseudo-myelomatous plasmacytosis of the bone marrow in a multicentric Castleman's disease].

The authors report the case of a 35 year-old polynesian male admitted to hospital with a diagnosis of myeloma. Bone marrow study showed an intense plasmocytosis, mature, Marshalko type. A new physical examination disclosed polyadenopathies and the lymph node biopsy showed lymph node modifications typical of plasma cell type Castleman's disease. At the same time, the polytypic profile of the bone marrow plasmacytosis and of the hypergammaglobulinemia was demonstrated, thus confirming the reactive state of the plasmacytosis, due to Castleman's disease. Such massive, reactive plasmacytosis belong to one of the various pathological features of multicentric Castleman's disease. Thus, this disease should belong to the etiologic diagnosis of intense bone marrow plasmacytosis and be distinguished from multiple myeloma, sometimes associated to Castleman's disease, especially in case of POEMS syndrome.

Adult↗

Successful treatment of severe hepatic veno-occlusive disease after allogeneic bone marrow transplantation by transjugular intrahepatic portosystemic stent-shunt (TIPS).

Severe veno-occlusive (VOD) disease is a major cause of morbidity and mortality in allogeneic bone marrow transplant recipients, and new approaches in managing patients who develop serious VOD is needed. In this report, we describe a patient who underwent transjugular intrahepatic portosystemic stent-shunt (TIPS) for life-threatening VOD are following allogeneic bone marrow transplantation for AML. Although the patient died of CMV-associated pneumonitis 5 weeks later, the TIPS functioned well until her death and permitted regression of the hepatic and renal symptoms. This report suggests that TIPS may be feasible and effective for managing patients with life-threatening liver dysfunction after marrow transplantation.

Adult↗

[Multiple myeloma and bone manifestations. Role of clodronate].

Osteolytic lesions in multiple myeloma are related to osteoclast activation induced by a network of cytokines including IL6, TNF beta or IL1 beta which explain pain, fractures, or hypercalcaemia appearance. Bone destruction is reflected by hydroxyprolinuria and inversely correlated with serum osteocalcin. Lytic lesions or osteopenia are present in 80% of patients on X-ray. MR imaging, is interesting for exploring spinal lesions or the so-called solitary plasmacytomas but still remains a tool in evaluation. After therapies few modifications are demonstrated on roetgenograms while osteodensitometry appears more sensitive. A review of the literature is made showing the prominent place of clodronate in the therapy of hypercalcaemia of multiple myeloma. This drug appears attractive for decreasing osteolysis and perhaps for improving quality of life of patients. Nevertheless, a better definition of its use in clinical practice is necessary.

Analgesics, Non-Narcotic↗

[Myelodysplasia and tuberculosis].

We report here the case of a patient with myelodysplasia and concomitant tuberculosis. Refractory anemia with blasts excess diagnosis was based upon morphological and cytogenetical criterias (del 20q), and tuberculosis was diagnosed on a cervical lymph node biopsy. Hematological data remained stable without any specific treatment for several months, cell counts even normalized under antituberculosis tritherapy. Clinical and hematological worsening appeared 3 years later, 1 year after discontinuation of antituberculosis therapy. It was characterized by progressive bone marrow failure and transformation in acute myeloid leukaemia. Concomitantly tuberculosis relapsed. The association of antituberculosis therapy and polychemotherapy (daunorubicine and aracytine) did not allow to obtain a hematological remission. The relationship between tuberculosis and myelodysplasia is discussed.

Anemia, Refractory, with Excess of Blasts↗

[Bladder neoplasms and cyclophosphamide. Apropos pf 3 cases amd review of the literature].

We report three new cases of bladder cancer occurring in patients treated by cyclophosphamide (Endoxan): two patients had Waldenström disease and were treated during 7.5 and 7 years respectively (total received dose of 220 and 190 g respectively). The third patient was treated for autoimmune erythroblastopenia and the bladder cancer occurred 5 years after treatment by cyclophosphamide (39 g during 3.3 years). Bladder cancers after cyclophosphamide treatment are generally transitional cell carcinomas. They are observed after an oral treatment, generally given for more than one year. A cumulative dose of more than 20 g is the principal risk factor, with a median interval from treatment to tumor of 7 years. No other risk factor has been identified (tobacco, age, sex, hemorrhagic cystitis). The relative risk of bladder cancer is estimated between 7 and 9, and seems proportional to the cumulative dose of cyclophosphamide. The first hypothesis to explain bladder cancer occurrence is a carcinogenic effect of one of the cyclophosphamide metabolites, acrolein, but the immunosuppressive effect of cyclophosphamide may play a role. The risk of secondary bladder cancer implies to limit the use of cyclophosphamide, particularly in non malignant disease, and to closely watch the patient especially by way of annual cystoscopy.

Aged↗

[Difference in costs of autologous transplantation of peripheral and bone marrow hematopoietic stem cells. A retrospective analysis over 1 year of transplantation in lymphoma, Hodgkin's disease and myeloma in a Center].

The study analysed the financial benefits of transplantation with peripheral blood stem-cells primed after chemotherapy and growth factor in comparison with bone marrow transplantation. Twenty-three consecutive transplantations were performed during one year: 14 peripheral blood stem-cell (CSC group) and 9 bone marrow transplantations (MO group). No differences in patients characteristics were seen between the two groups except for higher number of "BEAM" conditioning in CSC group. Were analyzed delay in neutrophil and platelet recovery, numbers of days in hospital, with fever, under antibiotics, costs of supportive therapy, stem-cell collection and cryopreservation. Difference was significant for duration of neutropenia with advantages in CSC group, but the number on days in hospital, with fever or under antibiotics were similar. Number of platelet transfusions was reduced in CFC group: this economical advantage was lost with the cost of growth factor used for priming stem-cells stem-cell collections and cryopreservations. In our retrospective study, financial advantages associated to peripheral blood stem-cell transplantation was not verified.

Adolescent↗

Overexpression of cyclin D2 in chronic B-cell malignancies.

Tumor progression in B-cell chronic lymphocytic leukemia (B-CLL) is thought to result from the gradual accumulation of small resting G0/G1 phase lymphoid cells rather than the proliferation of actively dividing cells. The recent identification of G1 cyclins that are likely to control both the progression through G0 and G1 phase and the G1/S transition prompted us to study the mRNA expression of D-type cyclins in the peripheral blood lymphocytes from 34 patients with B-CLL, 7 patients with lymphoplasmacytic lymphoma (LPL), and 2 patients with mantle cell lymphoma (MCL). Cyclin D2 mRNA was, on average, 5- to 10-fold overexpressed in most of the samples studied (B-CLL, 29/34; LPL, 7/7; MCL, 0/2) as compared with normal resting B lymphocytes, in which cyclin D2 mRNA was barely detectable. In situ hybridization with cyclin D2 digoxigenin-labeled mRNA probe showed that all the cells from a given sample were stained with approximately the same intensity. Cyclin D3 was never detected in any of the samples tested, whereas cyclin D1 was expressed in only the 3 cases (1 LPL and 2 MCL) bearing a t(11;14) translocation. A trisomy 12 was found in 4 of 19 (21%) B-CLL or LPL cases for which cytogenetic analysis was available. Although the cyclin D2 gene has been mapped to chromosome 12p13, there was no apparent correlation between trisomy 12 and the level of cyclin D2 expression. Cell cycle analysis by flow cytometry after staining with propidium iodide consistently showed that more than 96% of the cells were in G0/G1 phase, whatever the importance of cyclin D2 overexpression was, and that cyclin D2 overexpression in B-CLL was not associated with any modifications of the cell cycle repartition. No consistent overexpression of cyclin D2 was found in acute myeloid leukemias. In conclusion, overexpression of cyclin D2 mRNA was found to be an almost constant feature in B-CLL and LPL. Therefore, it led us to hypothesize, with the support of data from some transfection experiments previously reported in murine hematopoietic cell lines, that cyclin D2 might play a role in B-CLL pathogenesis, possibly by preventing cells from programmed cell death.

Cyclin D2↗

Small noncleaved cell lymphoma and leukemia in adults. A retrospective study of 65 adults treated with the LMB pediatric protocols.

In France, more than 80% of children with Burkitt's lymphoma or Burkitt's leukemia (ALL3) are now cured with the LMB (B-cell non-Hodgkin's lymphoma and B-ALL) protocols of the Société Française d'Oncologie Pédiatrique, but so far, poor results have been obtained in the few adult studies available. We have analyzed the experience with LMB protocols in adult patients. This retrospective study involved 65 adult patients with small noncleaved cell lymphoma or ALL3 treated with the LMB protocols. They were 17 to 65 years old and not previously treated. Human immunodeficiency virus-infected patients were excluded. The diagnoses were made between September 1984 and August 1991. According to the Murphy classification, 12 patients (18%) had stage I or II disease, 25 (38%), stage III disease; 4 (6%), stage IV disease; and 24 (37%), ALL3 (> or = 25% blasts). According to the Ann Arbor classification, 9 patients had stage I disease; 8 patients, stage II; 5 patients, stage III; 21 patients, stage IV disease; and 22 patients, ALL (> or = 30% blasts). Twelve patients had central nervous system (CNS) involvement before treatment. Thirty-nine patients were treated according to the LMB 84 protocol scheme; 14 according to the LMB 86 protocol, and 12 patients received the LMB 84 induction courses followed by the LMB 86 consolidation courses. Three patients underwent bone marrow transplantation (BMT) while in second complete remission (CR) and 3 others had refractory disease. There were some protocol violations caused by empirical medical decisions: local irradiation was performed in 4 patients, 2 patients received prophylactic radiation to the brain that was not specified in the protocol, 13 patients underwent BMT in first CR, and methotrexate doses were modified in 10 patients. Fifty-eight patients (89%) achieved a CR. There were four (6%) primary induction treatment failures, and three (4%) early treatment-related deaths. Eight patients relapsed between 2 and 30 months after CR (median, 4.7 months). Forty-seven patients are alive in CR (45 first CR, 2 second CR) with a median follow-up of 57 months (24 to 93 months). There were five toxicity-related deaths among patients in CR including four BMT-related deaths and five deaths caused by refractory relapses. One patient died in CR at 62 months of rectal cancer. The 3-year overall survival rate is 74% (SE = 5). According to the stages in the Murphy classification, the 3-year survival rates are stages I and II, 100%; stage III, 80% (SE = 7); and stage IV and ALL, 57% (SE = 8). Seven of 12 patients with initial CNS disease are alive with a median survival of 56 months.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗