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Biomedical subjects

F Maloisel

Publications and source records attributed to F Maloisel.

At least 37 records · Page 2Linked to original sources
PubMed2001

[Isolated neurologic disorders disclosing Biermer's disease in young subjects].

INTRODUCTION: In young subjects, the discovery of a cobalamine deficiency (Biermer's disease) at the time of neuropsychiatric disorders even of isolated MRI anomalies is a rare event (less than 0.01%). EXEGESIS: We report two observations of cobalamine deficiency, in patients 30 and 37 years old, revealed by acroparesthesia and spontaneous hypersignal in the T2 sequence of MRI and by a peripheral sensitive neuropathy respectively. CONCLUSION: The neuropsychiatric demonstrations of pernicious anemia are polymorphic with, sometimes, isolated spinothalamic attacks. The MRI can contribute to the diagnosis but more often leads to an erroneous diagnosis. Our observations are of special interest because symptoms occurred apart from supplementation of folates and recovered ad integrum with an early treatment by vitamin B12.

Adult↗
PubMed2001

Pancreatic metastasis of alveolar soft-part sarcoma: a case report and review of the literature.

Alveolar soft-part sarcoma is a rare tumour. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. We present a case of pancreatic metastasis, occurring more than six years after diagnosis. Treatment consisted in subtotal pancreatoduodenectomy with pylorus resection. Both specific patterns of relapse and treatment opportunities of this uncommon feature are discussed.

Adult↗
PubMed2001

Evaluating treatment strategies in advanced Waldenström macroglobulinemia: use of quality-adjusted survival analysis.

A randomized phase II multicenter clinical trial comparing the efficacy of fludarabine (FAMP) to that of the association of cyclophosphamide, doxorubicin and prednisone (CAP) in 92 patients with Waldenstrom's macroglobulinemia in first relapse or with primarily resistant disease, was conducted on the behalf of the 'Groupe Coopératif Macroglobulinémie'. The main analysis of this study failed to demonstrate a clear cut benefit of FAMP in terms of overall survival (OS), although a significant benefit in terms of time to disease progression and event-free survival (EFS) was noted. In this rare disorder, where few randomized trials have been conducted, we took advantage of this trial to assess treatment differences while integrating quality of life considerations. We thus performed a quality-adjusted survival analysis, using the quality-adjusted time without symptoms or toxicity (Q-TWiST) approach. Four health states differing in terms of quality of life (QoL) were defined, namely treatment-related toxicity, treatment free of toxicity, no treatment or symptoms, and relapse. The average time spent in these health states (TOX, CT, TWiST and REL, respectively) were then weighted by utility coefficients reflecting relative QoL value according to that of TWiST and summed up giving the so-called Q-TWiST. No difference was found between randomized groups in terms of mean CT. Mean TOX in the two groups were similarly close except when considering alopecia as a relevant toxic event. By contrast, mean TWiST was 5.9 months longer in the FAMP group than in the CAP group (P = 0.006). Unsurprisingly, given the absence of difference in OS but the difference in EFS in favor of the FAMP group, mean REL was increased by 6.8 months in the CAP group (P = 0.047). As a result, benefit of FAMP in terms of average Q-TWiST only relied on the value of the utility coefficient attributed to REL (U(REL)), with a significant benefit when UREL ranged from 0 to 0.28, ie in patients undergoing poor QoL after relapse, which is likely.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Otological manifestations of acute leukaemia: report of two cases and review of literature.

Otological manifestations exceptionally reveal acute leukaemia, whereas leukaemic infiltration of any tissue is frequent at postmortem examination. We present two cases of acute myeloblastic leukaemia revealed by a loss of hearing due to a middle-ear leukaemic infiltration. The characteristics of such a clinical and radiological finding are emphasized. It is suggested that middle and inner ear can be a 'sanctuary' localization, which might sometimes require radiation therapy to achieve durable and complete remission.

Adult↗
PubMed2001

Haematopoietic growth factor in antithyroid-drug-induced agranulocytosis.

Drug-induced agranulocytosis (DIA) is often caused by antithyroid drugs. We retrospectively studied the use of granulocyte colony-stimulating factor (G-CSF) therapy in antithyroid-DIA. Data for 20 patients (10 treated with G-CSF) with antithyroid-DIA (neutrophil count <0.5x10(9)/l) were extracted from a cohort study of DIA patients (n=110). G-CSF (300 microg/day subcutaneously) was used where the neutrophil count was <0.1x10(9)/l, or the patient was aged >70 years, or there were severe features of infection or underlying disease. Mean patient age was 62 years (range 34-87); sex ratio (M/F) was 0.05. Carbimazole (n=19) and benzylthiouracile (n=1) were the causative drugs, at mean doses of 30 mg/day (range 20-60) and 100 mg/day (range 50-150), respectively, for a mean of 37 days (range 31-90). Antithyroid drugs were prescribed for Graves' disease (n=8), thyrotoxicosis related to amiodarone intake (n=6) and multinodular goitre (n=6). Clinical features included isolated fever (n=7), pneumonia (n=5), septicaemia or septic shock (n=5) and acute tonsillitis (n=3). Mean neutrophil count was 0.07+/-0.1x10(9)/l. No patient died. Mean durations of haematological recovery, antibiotic therapy and hospitalization were significantly reduced with G-CSF: 6.8+/-4 days vs. 11.6+/-5; 7.5+/-3.8 days vs. 12+/-4.5; and 7.3+/-4.8 days vs. 13+/-6.1, respectively (all p<0.05). G-CSF induced flu-like symptoms in 30% of patients, but reduced overall costs.

Adult↗
PubMed2001

[Autoimmune peripheral neuropathies with anti-MAG antibodies and hematological disorders. Five cases].

OBJECTIVE: The aim of this study was to report our experience with autoimmune neuropathies associated with hematological disorders and to describe their etiological and clinical polymorphism. PATIENTS AND METHODS: A retrospective study was conducted in five patients with autoimmune peripheral neuropathies with anti-MAG (myelin-associated glycoprotein) antibodies. RESULTS: Autoimmune neuropathies were associated with Waldenström's macroglobulinemia (n=2), Hodgkin disease (n=1), chronic lymphocytic leukemia (n=1) and idiopathic polyclonal B lymphoproliferation (n=1). Most of the patients had a sensorial polyneuropathy, predominant in the legs, exhibiting slow progress. Our patients showed a disappointing response to chemotherapy with stabilization or short response.

Adult↗
PubMed2000

[Do hematopoietic growth factors have a role in the treatment of drug-induced agranulocytosis?].

PURPOSE: Our study was aimed at determining the role of hematopoietic growth factor in drug-induced agranulocytosis. METHODS: Fifty-five cases of drug-induced agranulocytosis were reviewed and subdivided retrospectively into a G-CSF group (n = 15) and an untreated group (n = 40). Mortality and hematological recovery (number of days required for neutrophil counts to exceed 1.5 x 10(9)/L) were studied in the two groups. RESULTS: The mean granulocyte count was 0.09 x 10(9)/L. All patients presented infection. In the G-CSF group, no mortality (0% versus 5%, P = 0.85) and a shorter recovery time (8.1 versus 9.5 days P = 0.39) were observed. No significant difference between the two groups was observed in either the duration of antibiotic therapy (9.3 days in the G-CSF group versus 10.1 days in the untreated group, P = 0.51) or duration of hospitalization (10 days in the treated group versus 11 days in the G-CSF group, P = 0.46). CONCLUSION: Our results as well as a literature review indicate that G-CSF could decrease the time to hematological recovery and perhaps reduce mortality. However, the exact role of hematopoietic growth factors requires further investigations.

Aged↗
PubMed2000

[Is there a place for interferon-alpha in the treatment strategy of multicentric Castleman's disease?].

INTRODUCTION: Castleman's disease is an unusual condition of unknown cause, consisting of massive proliferation of lymphoid tissue. Two forms (localized and multicentric) have been described. Interleukin-6 (IL-6) is at the core of the disease, being responsible for most of the clinical and biological signs that may be observed. Despite the benignancy of this pre-lymphoma condition, its course is usually aggressive and of poor prognosis in regard to the multicentric form. No consensus regarding treatment has been defined. Available data on the multicentric form of the disease are to scarce to allow any conclusion about the treatment timing and type of chemotherapy best suited to this condition. We report the case of a patient in whom interferon alpha (IFN-alpha) was used as first line treatment. EXEGESIS: The case of a 52-year-old man with multicentric Castleman's disease combined with high IL-6, in whom, however, testing for human herpes virus-8 proved to be negative, is described. Interferon alpha (4.5 MU/m2 three times per week during 18 months) administered as first line treatment induced dramatic improvement in the patient's general condition and normalization of the tumoral syndrome. Moreover, biological parameters and IL-6 returned to normal. Two years after interferon disruption, complete remission is still present. CONCLUSION: On the basis of the present data and those of two previous observations, anti-IL-6 and anti-infective properties of IFN-alpha are discussed. Treatment of multicentric Castleman's disease is based on corticosteroids and drugs derived from those pertaining to treatment of malignant lymphomas. Our results indicate that IFN-alpha is truly directed against Castleman's disease and has less toxicity than drugs usually prescribed. This argues for early use of IFN-alpha in Castleman's disease, in association or not with corticosteroids.

Castleman Disease↗
PubMed2000

Treatment of relapsing acute promyelocytic leukemia by all-trans retinoic acid therapy followed by timed sequential chemotherapy and stem cell transplantation. APL Study Group. Acute promyelocytic leukemia.

The purpose of this study was to assess the safety and efficacy of stem cell transplantation (SCT) mainly autologous SCT as consolidation therapy in APL patients who relapsed and achieved a second complete remission (CR2). Fifty adult patients with a first relapsed APL, of whom 39 had been previously treated with ATRA, entered a multicenter trial of oral ATRA until complete remission (CR) achievement followed by timed sequential chemotherapy (EMA combining etoposide 200 mg/m2/day for 3 days, mitoxantrone 12 mg/m2/day for 3 days, and cytarabine 500 mg/m2/day for two sequences of 3 days). EMA was started either after CR achievement, or on day 1 of ATRA because of initial white blood cell (WBC) counts >5 x 10(9)/l, or rapidly added to ATRA in order to prevent ATRA syndrome because WBC count increased under ATRA. Forty-five patients (90%, 95% CI 78%-97%) were in CR after induction therapy. Five patients died from infection during aplasia following EMA chemotherapy. Eleven patients who achieved CR had a familial HLA-identical donor and were allografted. The median disease-free survival (DFS) of allografted patients was 8.2 months. The 34 other CR patients were scheduled for autologous peripheral blood (PB) SCT (intent-to-treat group). Actually, autologous transplantation was only carried out in 22 patients (65%) (17 PBSCT and five autologous bone marrow transplantation (BMT)). Reasons for not autografting were early relapse (three patients), severe toxicity of EMA chemotherapy (six patients), and refusal or failure of stem cell harvest (three patients). The 3-year DFS rate of patients actually autografted was 77%. Among the 17 autografted patients still in CR2, nine patients have already reached a longer CR2 than first CR (CR1). Results of detection of PML/RARalpha by RT-PCR after autologous transplantation show negative findings in eight of the nine patients tested. We conclude that (1) ATRA combined to EMA chemotherapy is effective in the treatment of relapsed APL; (2) allogeneic BMT may be too toxic after salvage treatment including EMA intensive chemotherapy; (3) clinical outcome of autografted patients and preliminary molecular results regarding detection of PML/RARalpha after autologous PBSCT are encouraging.

Adult↗
PubMed2000

Long-term follow-up confirms the benefit of all-trans retinoic acid in acute promyelocytic leukemia. European APL group.

First results of a randomized trial (APL91 trial) and other randomized or non-randomized studies have shown that ATRA followed by chemotherapy significantly increased event-free survival (EFS) and survival, and decreased the incidence of relapse by comparison to chemotherapy alone in newly diagnosed APL. We present here long-term follow-up of the APL91 trial. In this trial, 101 patients had been randomized between ATRA followed by three courses of daunorubicin-AraC chemotherapy (ATRA group) and the same chemotherapy alone (chemotherapy group). Results were reanalyzed 73 months after closing of patient entry. Updated results of APL 91 trial found a Kaplan-Meier estimate of EFS and relapse rate at 4 years of 63% and 31% in the ATRA group, as compared to 17% and 78% in the chemotherapy group (P= 10(-4) and relative risk 2.95, P= 10(-4) and relative risk 3.68, respectively). Kaplan-Meier survival at 4 years was 76% in the ATRA group and 49% in the chemotherapy group (P= 0.026, relative risk 2.7). In the chemotherapy group, seven of the 27 relapses occurred after 18 months, but no relapse was seen after 43 months. In the ATRA group, four of the 17 relapses occurred after 18 months, including two late relapses (at 58 and 74 months). In the chemotherapy group, 23 of the 25 patients who relapsed achieved a second CR with ATRA, and the Kaplan-Meier estimate of second relapse was 40% at 30 months. In the ATRA group, the 10 patients who relapsed and were retreated with ATRA achieved a second CR. In conclusion, long-term results of APL91 trial confirm the superiority of the combination of ATRA and chemotherapy over chemotherapy alone in newly diagnosed APL, and that ATRA should be incorporated in the front-line treatment of APL.

Antineoplastic Agents↗
PubMed2000

Additional chromosomal abnormalities in patients with acute promyelocytic leukaemia (APL) do not confer poor prognosis: results of APL 93 trial.

In spite of the recent improvement in the outcome of acute promyelocytic leukaemia (APL) with treatment combining all trans retinoic acid (ATRA) and chemotherapy (CT), some patients with this disease still have a poor outcome. The prognostic significance of chromosomal abnormalities in addition to t(15;17) in APL is uncertain. We examined the prognostic significance of secondary chromosomal changes in 292 patients included in a European trial who were treated with ATRA and CT. The incidence of chromosomal abnormalities in addition to t(15;17) was 26% and trisomy 8 was the most frequent secondary change (46% of the cases with secondary changes). No significant differences were seen with regard to age, sex, initial white blood cell count, % of circulating blasts, platelet count, fibrinogen level and incidence of microgranular variants between patients with or without additional rearrangements. Outcome was also similar between patients with t(15;17) alone and patients with t(15;17) and other clonal abnormalities for complete remission (92% vs. 93% respectively), event-free survival at 2 years (76.1% vs. 78.1% respectively), relapse at 2 years (16.7% vs. 11.6% respectively) and overall survival at 2 years (79.9% vs. 79.5% respectively). Analysis according to the type of induction treatment (ATRA followed by CT or ATRA plus CT) or the type of maintenance treatment (with ATRA, low-dose CT or both) also failed to show any difference between the two groups. Thus, in a large cohort of APL patients treated with ATRA and CT, additional chromosomal abnormalities had no impact on prognosis.

Adult↗
PubMed2000

[Failure of bone marrow autograft in metastatic malignant adrenalcortical carcinoma].

BACKGROUND: Despite different therapeutic modalities (surgery, hormones therapy, chemotherapy), 5-year survival in patients with malignant adrenocortical carcinoma remains only 10% to 20%. Bone marrow grafts may be proposed to intensify treatment. CASE REPORT: In a 29-year-old patient with adrenocortical carcinoma with metastases to the lung and bone, an adjuvant bone marrow graft using peripheral stem cells was unsuccessful. Recurrence was observed at 3 months and overall survival was 2 years. DISCUSSION: Our case illustrates the feasibility of this approach but also emphasizes the relatively poor results obtains with only 2 years survival (1 year after the bone marrow graft), comparable with other classical modalities reported in the literature.

Adrenal Cortex Neoplasms↗
PubMed1999

[Therapeutic aspects in the management of hairy cell leukemia].

CHRONIC B CELL PROLIFERATION: An estimated 80 to 120 new cases of hairy cell leukemia are diagnosed annually in France. Median survival is 5 years for untreated patients who develop a series of infectious complications. For many years, interferon alpha was the standard therapy but the therapeutic strategy has changed with the arrival of purine analogs, deoxycoformicine and 2-CdA. THERAPEUTIC OPTIONS: We searched Medline, Pascal, and Current Contents for literature on the treatment of hairy cell leukemia over the last 10 years and discuss here available data on response rate, mechanism of action and adverse effects of different therapeutic options. BY TREATMENT: Interferon generally induces partial response and most patients relapse after treatment withdrawal. The purine analogs, desoxycoformycine and 2-chlorodeoxyadenosine, are more active than interferon inducing response in approximately 90% of the cases, even after interferon failure, complete response is achieved in 50% to 70% of patients. Relapse rate at 5 years appears to be limited to 10% n 15%. Besides infections, the main adverse effect is the constant deep and persistent decline in CD4 counts but with no special risk of opportunistic infection. The increased rate of secondary cancers in long-term survivors and its possible relationship with treatments remains a controversial topic.

Antibiotics, Antineoplastic↗