Biomedical subjects
C Cordonnier
Publications and source records attributed to C Cordonnier.
[Cellular neurothekeoma, a rare cutaneous tumor. Anatomo-clinical and immunohistochemical study of 2 cases].
Cellular neurothekeoma is an unusual cutaneous tumor described in 1986. We report two new cases in 14-year-old girls. Both tumors measured less than one centimeter and were located on the forearm and the shoulder. They had a plexiform architecture and were developed in the dermis and the dermo-hypodermic junction. Spindle and epithelioid tumor cells were immunoreactive for vimentin, NKI-C3, NSE, CD68 and smooth muscle actin. There was no recurrence 2 and 7 months after the operation. Cellular neurothekeoma is a benign tumor. Its histogenesis is still unknown. Histological and immunohistochemical features, with positivity of the tumor cells for NKI-C3, a non specific melanocytic marker, and negativity for S-100 protein and HMB-45, allow to differentiate cellular neurothekeoma from myxoid neurothekeoma and melanocytic tumors.
[Mesothelial cyst of the adrenal gland].
We report a case of a mesothelial adrenal cyst which was incidentally discovered in a 38-year old woman. The diagnosis was confirmed by an immunohistochemistry study. Adrenal cysts are rare. They occur more commonly in female patients during the 4th and 5th decades and are usually asymptomatic. Adrenal cysts are divided into four groups : parasitic, epithelial, endothelial cysts and pseudocysts. The management is still discussed : follow-up, surgical resection or fine-needle aspiration.
[Diagnosis with hair].
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[Plurifocal testicular hamartomas and testicular feminization syndrome].
We report the case of a 14-year-old girl with a testicular feminization syndrome. The inguinal cryptorchid testis contained plurifocal hamartomas ranging from 0.5 to 1 cm. They were composed of tubules lined by cylindrical Sertoli cells immunoreactive for alpha-inhibin and p30/32(MIC2). The stroma contained few Leydig cells. Ultrastructural study showed tubules with immature Sertoli cells. The testicular feminization syndrome is caused by mutations of the androgen receptor gene. Patients with male genotype 46, XY have a female morphotype with external sexual organs without ambiguity. They have neither uterus nor ovary but two cryptorchid testis in which sex-cord stromal tumors can develop. Their malignant transformation is rare but requires preventive bilateral orchidectomy.
Quinine improves results of intensive chemotherapy (IC) in myelodysplastic syndromes (MDS) expressing P-glycoprotein (PGP). Updated results of a randomized study. Groupe Français des Myélodysplasies (GFM) and Groupe GOELAMS.
We designed a randomized trial of IC with or without quinine, an agent capable of reverting the multidrug resistance (mdr) phenotype, in patients aged < or = 65 years with high risk MDS. Patients were randomized to receive Mitoxantrone 12 mg/m2/d d2-5 + AraC 1 g/m2/12 h d1-5, with (Q+) or without (Q-) quinine (30 mg/kg/day). 131 patients were included. PGP expression analysis was successfully made in 91 patients and 42 patients (46%) had positive PGP expression. In PGP positive cases, 13 of the 25 (52%) patients who received quinine achieved CR, as compared to 3 of the 17 (18%) patients treated with chemotherapy alone (p = 0.02). In PGP negative cases, the CR rate was 35% and 49%, respectively in patients who received quinine or chemotherapy alone (difference not significant). In the 42 PGP positive patients, median Kaplan-Meier (KM) survival was 13 months in patients allocated to the quinine group, and 8 months in patients treated with chemotherapy alone (p = 0.01). In PGP negative patients, median KM survival was 14 months in patients allocated to the quinine group, and 14 months in patients treated with chemotherapy alone. Side effects of quinine mainly included vertigo and tinnitus that generally disappeared with dose reduction. Mucositis was significantly more frequently observed in the quinine group. No life threatening cardiac toxicity was observed. In conclusion, results of this randomized study show that quinine increases the CR rate and survival in PGP positive MDS cases treated with IC. The fact that quinine had no effect on the response rate and survival of PGP negative MDS suggests a specific effect on PGP mediated drug resistance rather than, for instance, a simple effect on the metabolism of Mitoxantrone and/or AraC.
Lack of evidence of heterozygosity for cystic fibrosis as a risk factor for invasive aspergillosis in haematological patients.
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[Splenic angiosarcoma: a case report with synchronous hepatic metastases].
A 49 years old man in good physical condition suffering from abdominal pain in the right hypochondrium was hospitalized and CT scan revealed an hemoperitoneum, an enlargement of the liver and a rupture of an heterogeneous spleen. The patient was operated on and spleen removed. Pathological examination of the spleen concluded to a splenic cavernous hemangioma. In the postoperative course, an increase of liver nodules occurred within two weeks. A localized splenic angiosarcoma was recognized by reexamination of the specimen. The patient treated by chemotherapy was still alive after 5 months. Prognosis of splenic angiosarcoma is very poor; there is no curative treatment.
Granulocyte-macrophage colony-stimulating factor (GM-CSF) to increase efficacy of intensive sequential chemotherapy with etoposide, mitoxantrone and cytarabine (EMA) in previously treated acute myeloid leukemia: a multicenter randomized placebo-controlled trial (EMA91 Trial).
The EMA86 study showed efficacy of intensive sequential chemotherapy with mitoxantrone, 12 mg/m2 day on days 1-3, etoposide, 200 mg/m2/day as a continuous infusion on days 8-10 and cytarabine (araC), 500 mg/m2/day as continuous infusion on days 1-3 and 8-10 (EMA regimen) in previously treated patients with AML. The goal of the EMA91 study was to determine whether administration of GM-CSF between the two sequences of EMA chemotherapy and during the second sequence could increase therapeutic efficacy by potentially increasing leukemic cell recruitment into the S phase of cell cycle before the second sequence. One hundred and ninety-two patients aged less than 65 years with previously treated AML received GM-CSF, 5 microg/kg/day or placebo from day 4 to day 8 of EMA chemotherapy. One hundred and twenty were refractory and 72 were in first relapse after a complete remission (CR) of more than 6 months duration. CR rates after one course of chemotherapy were 65% in the GM-CSF group (refractory: 51%; first relapse: 89%), not significantly different from the 59% CR rate (refractory: 46%; first relapse: 81%) in the placebo group. Median time to recovery of neutrophils was 38 and 37 days and median time to last platelet transfusion 32 and 32 days respectively in the GM-CSF and placebo groups. WHO grade > or = 3 non-hematologic toxicities were mainly sepsis (45% and 51%, respectively) and mucositis (34% and 31%) and did not differ between the two groups. Toxic death rate was 5% and 8%, respectively, in the GM-CSF and placebo groups. Patients achieving CR were scheduled to receive six courses of maintenance with reduced-dose EMA. Time to progression tended to be longer in the GM-CSF group (median 154 vs 115 days, progression-free rate at 18 months 33% vs 19%, P = 0.08), particularly in refractory patients (P = 0.06). However, at the current follow-up, this did not translate into a significantly longer disease-free survival and survival. Cell cycle studies showed increased recruitment of cells in the S phase between day 4 and day 8 in the GM-CSF group compared to placebo (P = 0.006). However, this did not significantly relate to prognosis in this cohort of patients. GM-CSF might marginally increase efficacy of sequential chemotherapy without increasing its toxicity in the absence of any detected relationship between this effect and observed leukemic cell recruitment into the cell cycle.
Usefulness of genotyping with microsatellite markers to investigate hospital-acquired invasive aspergillosis.
To assess whether invasive aspergillosis (IA) was hospital acquired, Aspergillus fumigatus isolates, obtained during a one-year study from inpatients with haematological diseases and IA and from their environment, were genotyped using microsatellite markers. The analysis of 62 environmental isolates showed an extremely diverse A. fumigatus population with 43 genotypes represented only once. Eight genotypes were found more than twice at different times and/or at different locations showing that a given isolate can persist over time and is not dependent on a specific location. Twenty-seven isolates were obtained from 12 patients with IA. Of eight patients with multiple isolates, four were infected with isolates of different genotypes. Five patients (42%) had hospital-acquired IA according to the following definitions: patients infected with an isolate found in the environment, or patients infected with the same genotype. Although genotyping results are highly suggestive of hospital-acquired IA, this cannot be proved definitively because of the high diversity of the A. fumigatus population and the limited environmental sampling. A better knowledge of the A. fumigatus population outside hospitals is needed. For this purpose, genotyping using microsatellite markers seems appropriate.
Bacteremia due to Capnocytophaga species in patients with neutropenia: high frequency of beta-lactamase-producing strains.
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[Papillary fibroelastoma. Report of 3 cases].
Papillary fibroelastoma is a rare, benign, primary cardiac tumour. The authors report three cases presenting with transient or full cerebrovascular accidents. The tumours were diagnosed during investigation by echocardiography, especially transoesophageal echocardiography. In view of the severity of symptoms and the high risk of recurrence, the patients were rapidly referred for surgery. In two cases, the tumour was located on the aortic valve at several sites. In one case, the tumour was single and implanted on the mitral valve. Surgery was successful with the pathology reports confirming complete ablation. The patients have not had any recurrence to date. The multiple nature of these tumours raises the questions of local spread, secondary degeneration of valvular nodes? The problem of possible recurrences is discussed.
Reverse seroconversion of hepatitis B after allogeneic bone marrow transplantation: a retrospective study of 37 patients with pretransplant anti-HBs and anti-HBc.
BACKGROUND: Reverse seroconversion to hepatitis B virus (HBV), i.e., HBV reactivation in patients with pretransplant antibodies to hepatitis B surface antigen (anti-HBs) and to hepatitis B core antigen (anti-HBc), is rarely re-ported after allogeneic bone marrow transplantation. METHODS: To determine this risk, we studied clinical outcome and serological changes in 37 patients with pretransplant anti-HBs and anti-HBc. RESULTS: In 33 cases, no change in HBV markers was observed in the posttransplant period. In four cases, anti-HBs and anti-HBc were lost, and hepatitis B surface antigen, hepatitis B e antigen, and HBV DNA emerged together with acute hepatitis, after cessation of immunosuppression. The actuarial risk of reactivation in the 37 patients was 20.5% (median follow-up 20 months). No reactivation occurred in patients with anti-HBs-positive donors. CONCLUSION: Although few cases of postallogeneic bone marrow transplantation reverse seroconversion to HBV have been reported, this study demonstrates that the actuarial risk is relatively high and suggests that donor vaccination might be proposed prophylactically or that HBs-specific immunoglobulin infusions might be warranted.
Human herpesvirus 6 infection in patients with exanthema after allogeneic bone marrow transplantation.
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An EORTC multicentre prospective survey of invasive aspergillosis in haematological patients: diagnosis and therapeutic outcome. EORTC Invasive Fungal Infections Cooperative Group.
OBJECTIVES: The EORTC Invasive Fungal Infections Cooperative Group (IFICG) conducted a prospective survey by questionnaire of all cases of invasive aspergillosis (IA) in cancer patients to ascertain current diagnostic and therapeutic approaches. METHODS: All members of the IFICG were asked prospectively to complete a detailed questionnaire for each IA case identified in their institution over a 12-month period. RESULTS: One hundred and thirty questionnaires were returned. All cases were independently evaluated (DWD & JC) and 123 were eligible. Cases came from 20 hospitals in eight countries and the number of cases per institution varied from 1-21. Acute myeloid leukaemia (AML) (60, 49%), acute lymphoblastic leukaemia (ALL) (21, 17%) and lymphoma (11, 9%) were the most frequent underlying diseases, and 16 (12%) patients had received an allogeneic bone marrow transplant. Pulmonary involvement was present in 87%, infection of sinuses/nose in 16% and brain in 8%. The chest radiograph was initially normal in 9% of those with primary pulmonary disease. The diagnosis was confirmed in 50%, probable in 31% and possible in 19%. The evidence for IA was on the basis of clinical and radiological features alone in 28%, with culture or histology in another 31% and 9%, respectively, and with both culture and histology in 29%. In three (2%) patients with diagnosis was based on culture or histology alone. Treatment was given to 120 patients (98%)-amphotericin B 75%, lipid-associated amphotericin B 36%, itraconazole 40%, flucytosine 12%, growth factors 33%, lobectomy 5%. At 3 months after diagnosis or first suspicion of IA, 44 (36%) patients were alive and 79 (64%) dead. Outcome was best in those with AML (30% death and 46% with a complete antifungal response or cure). Growth factors (mostly granulocyte colony stimulating factor) appeared not to influence outcome (P = 0.99). CONCLUSION: IA remains a considerable diagnostic and therapeutic challenge. No single diagnostic procedure was universally successful and a multifaceted approach including surgery is necessary. There was no discernable difference in outcome between initial therapy with amphotericin B, itraconazole or lipid-associated amphotericin B, although numbers are limited and the study was retrospective.
The role of mediastinoscopy in the diagnosis of mediastinal lymphadenopathy.
OBJECTIVE: The reported experience of axial mediastinoscopy (MDS) performed in a diagnostic purpose only (rather than prognostic) is limited. Therefore, we designated the present study to clarify morbidity, sensitivity and accuracy of MDS performed to diagnose various mediastinal lesions. METHODS: We prospectively performed 400 MDS in a diagnostic purpose on 398 patients for: (1) isolated mediastinal adenopathies in 271 patients (group 1), and (2) mediastinal adenopathies associated with a pulmonary or a hilar lesion of unknown aetiology in 127 patients (group 2). In group 1, most of the patients were suspected to have a sarcoidosis, a tuberculosis or a lymphoma. In group 2, most of the patients were suspected to have a lung cancer. In both groups, the other current diagnostic procedure usually used in each pathology had failed to give an accurate diagnosis. RESULTS: A total of 76% of the samples were performed in the right laterotracheal lymph node station, 12.5% in the lower subcarinal and superior subcarinal lymph node station and 7.8% in the left laterotracheal lymph node station. The per- and post-operative mortality rates were nil. The per-operative morbidity accounted for six cases (1.5% of the examinations). The post-operative morbidity accounted for three cases (0.75% of the examinations). MDS data radically modified the pre-operative suspected diagnosis in 74 patients (18.5% of the patients). There were 17 false negative results (4.3% of the patients). The global sensitivity of MDS was 94%, the global specificity was 100% and the accuracy was 95%. In group 1, the sensitivity was 96% and in group 2 it was 92%. CONCLUSION: According to the results, the few contraindications of the procedure and its low cost, we confirm that MDS is still the first choice procedure to diagnose lesions located in the axial mediastinum.
Use of intravenous immune globulin in addition to antiviral therapy in the treatment of CMV gastrointestinal disease in allogeneic bone marrow transplant patients: a report from the European Group for Blood and Marrow Transplantation (EBMT). Infectious Diseases Working Party of the EBMT.
The best treatment of CMV gastrointestinal disease has been controversial, with some centers adding intravenous (i.v.) Ig to antiviral chemotherapy. The aim of this retrospective survey was to compare the outcome of antiviral chemotherapy with or without i.v. Ig. A questionnaire was sent to centers belonging to the EBMT. Thirty-three patients with CMV gastrointestinal disease were reported, 22 patients were given antiviral chemotherapy alone and 11 patients a combination of antiviral chemotherapy and i.v. Ig. Eighteen of 33 (55%) patients responded to therapy, 13 of those treated with antiviral chemotherapy alone and five (45%) of those treated with the combination (P = NS). Patients with acute GVHD of grades II-IV had significantly worse outcomes than patients with acute GVHD grades 0-I. In a Cox proportional hazards model corrected for acute GVHD there was no difference in outcome of CMV gastrointestinal disease with or without addition of Ig. Survival at 100 days after diagnosis of CMV gastrointestinal disease was 64%. There was no difference in survival in patients treated with or without i.v. Ig. The results of this retrospective survey indicate that addition of i.v. Ig to antiviral chemotherapy might not improve outcome in patients with biopsy-proven CMV gastrointestinal disease.
Quinine improves the results of intensive chemotherapy in myelodysplastic syndromes expressing P glycoprotein: results of a randomized study.
Intensive chemotherapy produces a lower complete remission (CR) rate in the myelodysplastic syndromes (MDS) than in de novo acute myeloid leukaemia (AML), possibly due in part to a higher incidence of P glycoprotein (PGP) expression in MDS blast cells. We designed a randomized trial of intensive chemotherapy with or without quinine, an agent capable of reverting the multidrug resistance (mdr) phenotype, in patients aged < or = 65 years with high-risk MDS. Patients were randomized to receive mitoxantrone 12 mg/m2/d days 2-5 + AraC 1 g/m2/12 h days 1-5, with (Q+) or without (Q-) quinine (30 mg/kg/d). 131 patients were included. PGP expression analysis was successful in 91 patients. In the 42 PGP-positive cases, 13/25 (52%) patients in the Q+ group achieved CR, compared to 3/17 (18%) patients in the Q- group (P = 0.02) and median Kaplan-Meier survival was 13 months in the Q+ group, and 8 months in the Q- group (P = 0.01). No life-threatening toxicity was observed with quinine. In conclusion, the results of this randomized study show that quinine increases the CR rate and survival in PGP-positive MDS cases treated with intensive chemotherapy.