525 results on '"P. Bordigoni"'
Search Results
102. Infantile osteopetrosis: a case report on dental findings
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D. Droz-Desprez, Martine Bonnaure-Mallet, P. Bordigoni, and C. Azou
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Male ,Cancer Research ,medicine.medical_specialty ,Tooth eruption ,Root Resorption ,Dentistry ,Unerupted tooth ,Dental Caries ,Pathology and Forensic Medicine ,Tooth Eruption ,stomatognathic system ,Oral and maxillofacial pathology ,Medicine ,Humans ,Tooth, Unerupted ,Child ,Bone Marrow Transplantation ,Gubernaculum ,business.industry ,Older brother ,Osteopetrosis ,Enamel hypoplasia ,medicine.disease ,Surgery ,stomatognathic diseases ,surgical procedures, operative ,Graft-versus-host disease ,Otorhinolaryngology ,Tooth Diseases ,Periodontics ,Odontogenesis ,Oral Surgery ,business - Abstract
In this paper we have described the case of a 7-yr-old Moroccan osteopetrotic boy, who had received a bone marrow transplant (BMT). He was transplanted from his older brother and, despite immunosuppressive therapy, developed chronic graft-versus-host disease and was placed on corticotherapy. Seven months after the bone marrow transplant, graft versus host disease (GVHD) was stabilized, but corticotherapy had inhibited growth. There was evidence of normalizing bone, his hearing was better but he had not recovered vision. Dental findings before the bone marrow transplant revealed some missing teeth, failure of teeth to erupt and decayed teeth but no enamel hypoplasia. The patient had developed one carious lesion on one unerupted tooth: bacteria seem to have found a way through the gubernaculum dentale. The scanning electronmicrographs showed decayed tooth and tissues fitted into each other. Since the bone marrow transplant, no tooth has erupted. We think that, in this case, failure of tooth eruption would be the sign of osteopetrosis. more...
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- 1992
103. [False forms of cardiolipin syndrome. Apropos of a case]
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C, Schmitt, A M, Worms, D, Sommelet, P, Bordigoni, B, Peiffert, G, Faure, and B, Teissert
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Diagnosis, Differential ,Male ,Antibodies, Anticardiolipin ,Humans ,Thrombosis ,Blood Coagulation Tests ,Femoral Vein ,Iliac Vein ,Antiphospholipid Syndrome ,Child ,Arthritis, Juvenile - Published
- 1992
104. Chronic gvhd is increased after allo blood cell transplantation but is associated with a reduction of relapse rate
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D. Blaise, M. Kuentz, J.H. Bourhis, N. Milpied, L. Sutton, J.P. Jouet, M. Attal, P. Bordigoni, J.Y. Cahn, J.M. Boiron, and M.Michallet for the SFGM
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Cancer Research ,medicine.medical_specialty ,business.industry ,Cell Biology ,Hematology ,Relapse rate ,medicine.disease ,Gastroenterology ,law.invention ,Surgery ,Blood cell ,Transplantation ,Leukemia ,medicine.anatomical_structure ,Randomized controlled trial ,law ,Internal medicine ,Genetics ,medicine ,Chronic gvhd ,Stage (cooking) ,business ,Molecular Biology - Abstract
We recently reported the results of a randomized study comparing allo BMT (N = 53) and BCT (N = 48) (JCO, Vol. 18, N.3, Feb 2000) for early stage leukemia: this study establishes that BCT is associated with better hematological recovery, lower costs, no more AGVHD and identical outcome. In the present analysis, with a longer follow-up (27 mths (13-41)), we focus on cGVHD. cGVHD is more frequent after BCT (28/48 (58%) vs. 16/53 (30%) (p more...
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- 2000
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105. Single or double consolidation treatment according to remission status after initial therapy in metastatic neuroblastoma: first results of LMCE 3 study in 40 patients
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J M, Zucker, T, Philip, J L, Bernard, J, Michon, E, Bouffet, J C, Gentet, M, Lopez, C, Coze, I, Philip, and P, Bordigoni
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Male ,Neuroblastoma ,Double-Blind Method ,Child, Preschool ,Antineoplastic Combined Chemotherapy Protocols ,Granulocyte-Macrophage Colony-Stimulating Factor ,Humans ,Female ,Prognosis ,Bone Marrow Transplantation ,Follow-Up Studies ,Neoplasm Staging ,Probability - Published
- 1991
106. The role of BMT for neuroblastoma relapse patients. A report of the EBMT-STR
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R, Ladenstein, F, Chauvin, A, Garaventa, J L, Bernard, J M, Zucker, P, Lutz, P, Bordigoni, E, Plouvier, J Y, Cahn, and D, Frappaz
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Europe ,Reoperation ,Neuroblastoma ,Antineoplastic Combined Chemotherapy Protocols ,Remission Induction ,Humans ,Neoplasm Metastasis ,Neoplasm Recurrence, Local ,Prognosis ,Combined Modality Therapy ,Survival Analysis ,Bone Marrow Transplantation - Published
- 1991
107. The LMCE1 unselected group of stage IV neuroblastoma revisited with a median follow up of 59 months after ABMT
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T, Philip, J M, Zucker, J L, Bernard, P, Lutz, P, Bordigoni, E, Plouvier, A, Robert, H, Roché, G, Souillet, and E, Bouffet
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Male ,Neuroblastoma ,Child, Preschool ,Antineoplastic Combined Chemotherapy Protocols ,Remission Induction ,Humans ,Transplantation, Homologous ,Female ,Transplantation, Autologous ,Bone Marrow Transplantation ,Follow-Up Studies ,Neoplasm Staging - Published
- 1991
108. The TAM regimen prior to allogeneic and autologous bone marrow transplantation for high-risk acute lymphoblastic leukemias: a cooperative study of 62 patients
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J Y, Cahn, P, Bordigoni, G, Souillet, J L, Pico, E, Plouvier, J, Reiffers, E, Benz-Lemoine, J P, Bergerat, P, Lutz, and P, Colombat
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Adult ,Adolescent ,Cytarabine ,Pilot Projects ,Precursor Cell Lymphoblastic Leukemia-Lymphoma ,Combined Modality Therapy ,Survival Analysis ,Transplantation, Autologous ,Child, Preschool ,Antineoplastic Combined Chemotherapy Protocols ,Humans ,Transplantation, Homologous ,Child ,Melphalan ,Whole-Body Irradiation ,Bone Marrow Transplantation - Abstract
A total of 62 patients with high-risk acute lymphoblastic leukemia (ALL) were treated with fractionated total body irradiation, high-dose cytosine arabinoside and melphalan followed by bone marrow transplantation (BMT). Thirty-six patients received allogeneic and 26 autologous BMT. Eight patients were treated in CR1, 36 in CR2 (first relapse occurring on therapy for 32), seven in further CR, 10 in relapse (five early first relapse, four second relapse and one fourth relapse) and one with refractory ALL. Severe toxicity occurred in 26 of the 62 patients (42%) and 14 died (22.5%) from non-leukemic causes. The actuarial event-free survival at 3.6 years was 28% after autologous BMT and 52% after allogeneic BMT with actuarial relapse rates of 62% and 35%, respectively. The results of this pilot study seem promising for this group of poor risk ALL, but the relapse rate remains high after autologous BMT and needs to be improved. more...
- Published
- 1991
109. ['Pseudo-lupus' eruptions in a mother carrying X chromosome-linked chronic septic granulomatosis]
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J F, Cuny, F, Chauvel, J L, Schmutz, P, Bordigoni, M, Weber, and J, Beurey
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Adult ,Diagnosis, Differential ,Male ,X Chromosome ,Lupus Erythematosus, Cutaneous ,Humans ,Infant ,Female ,Hand Dermatoses ,Granulomatous Disease, Chronic ,Facial Dermatoses - Abstract
We report the case of a young woman whose son had X-linked chronic granulomatous disease (CGD) while she was gene transmitter and presented with erythematous-squamous dermatosis predominant in unprotected regions, photosensitivity and oral ulcerations. The diagnosis of discoid lupus erythematosus, suggested by the clinical complex, was not confirmed by paraclinical examinations. CGD is an X-linked or, less often, autosomal recessive disease underlain by a selective deficiency of intraleucocytic bactericidal ability. Its clinical manifestations are repeated and severe infections involving most of the body organs. A review of the literature yielded 20 cases resembling that of our patient; all concerned mothers or sisters of children with X-linked CGD. The skin disease usually begins during childhood. The authors describe cutaneous manifestations as a photosensitive infiltrating erythema, frequently suggestive of lupus erythematosus, and oral ulcerations. At light microscopy, these skin lesions show a lymphohistiocytic infiltrate which sometimes erodes the basement membrane or remains at a distance from it. Such images suggest lupus erythematosus or, for some authors, a Jessner-Kanoff syndrome or a lichenoid infiltrate. Direct immunofluorescence was negative in all but one case, and there was usually no laboratory evidence of autoimmunity. In women who transmit the X-linked form of the disease, the bactericidal activity of granulocytes is reduced by 50 p. 100, but infection is not a major problem. The pathogenesis of these disorders is not yet fully understood. However, we would like to draw attention to the studies by Roberts et al. who demonstrated a defective degradation of bacterial DNA by circulating monocytes in patients with systemic or discoid lupus erythematosus.(ABSTRACT TRUNCATED AT 250 WORDS) more...
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- 1990
110. 714 Un cas d’uvéite liée à une granulomatose septique liée à l’X
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F. Rossi, T. Maalouf, J.L. George, P. Bordigoni, Karine Angioi, and C. Pecheur
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Ophthalmology - Abstract
But Rapporter un cas d’uveite liee a une granulomatose septique liee a l’X. Objectifs et Methodes Etude d’un cas d’uveite chez un enfant dont le diagnostic de granulomatose septique chronique a ete concomitant. Observation Un garcon de 2 ans, atteint d’un syndrome infectieux febrile severe depuis un mois, est adresse pour œil rouge. L’examen retrouve un tyndall de chambre anterieure unilateral droit a deux croix, des petits amas iriens blancs et des synechies irido-cristalliniennes, rendant le fond d’œil inaccessible. Une endophtalmie endogene est evoquee et traitee par antibiotherapie. Une pleuropneumopathie fongique, associee a une alteration des polynucleaires neutrophiles, fait suspecter aux pediatres une granulomatose septique chronique confirmee par l’analyse genetique du chromosome X qui designe une mutation sur l’ARN messager touchant la sous-unite gp 91phox du cytochrome b558. L’enfant est traite par une antibiotherapie mixte et des antifongiques. Malgre ce traitement, l’œil reste tres rouge et les amas iriens ont conflue en une masse blanc jaunâtre de la chambre anterieure pouvant faire evoquer un granulome. Une corticotherapie orale est mise en place. L’etat oculaire s’ameliore avec une disparition de l’inflammation et de la masse irienne. L’acces au fond d’œil reste impossible. Discussion La granulomatose septique chronique est un deficit immunitaire rare caracterise par des infections graves et repetees avec des foyers infectieux granulomateux cutanes ou ganglionnaires et des localisations viscerales diverses. C’est une maladie hereditaire a transmission autosomique recessive ou recessive liee a l’X(70 % des cas). Elle est liee a une incapacite des phagocytes a tuer les microorganismes par endocytose, due a une NADPH oxydase absente ou non fonctionnelle. Le test diagnostique est la mesure de la reduction du nitrobleu de tetrazolinium. Le traitement repose sur une prophylaxie antimicrobienne et fongique au long cours. L’atteinte oculaire la plus frequente est la presence de lesions chorioretiniennes le long des arcades vasculaires epargnant la macula ainsi que des keratites marginales et des blepharo-conjonctivites. Conclusion La particularite de cet enfant atteint de granulomatose septique chronique est l’atteinte oculaire du segment anterieur a type de granulome. more...
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- 2007
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111. Syndrome néphrotique et dysplasie spondyloépiphysaire :Dysplasie immuno-osseuse de Schimke
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M.J. Krier, B. Leheup, P. Bordigoni, J.L. André, and N. Cheurfa
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Pediatrics, Perinatology and Child Health - Published
- 1998
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112. TO68 - Les traitements opioïdes pour les douleurs par excès de nociception en oncologie pédiatrique
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A. Salmon, L. Mansuy, E. Pichard, L. Clément, P. Bordigoni, Claudine Schmitt, and F. Fouyssac
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Anesthesiology and Pain Medicine ,Neurology (clinical) - Published
- 2006
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113. ONCO4 Imagerie des formes viscerales graves de la mucormycose chez 5 patients immunodeprimes
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M. Sheibanifar, A.L. Guérin, L. Clément, J. Champigneulle, P. Bordigoni, and M. Claudon
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Radiological and Ultrasound Technology ,Radiology, Nuclear Medicine and imaging - Published
- 2006
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114. Comparaison de l'évolution de deux séries de gliomes du tronc cérébral sur une période de 28 ans. Étude unicentrique de 74 cas
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Pascal Chastagner, P. Bordigoni, F Plénat, P Lefevre, D. Sommelet, J. C. Marchal, S. Bracard, and S Hoffstetter
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Pediatrics, Perinatology and Child Health - Published
- 1997
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115. A Stochastic Control Approach to a Robust Utility Maximization Problem.
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Benth, Fred Espen, Nunno, Giulia, Lindstrøm, Tom, Øksendal, Bernt, Zhang, Tusheng, Bordigoni, Giuliana, Matoussi, Anis, and Schweizer, Martin
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- 2007
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116. Exclusion of Patients with a Severe T-Cell Defect Improves the Definition of Common Variable Immunodeficiency
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Bertinchamp, Rémi, Gérard, Laurence, Boutboul, David, Malphettes, Marion, Fieschi, Claire, Oksenhendler, Eric, Oksenhendler, E., Fieschi, C., Malphettes, M., Galicier, L., Boutboul, D., Fermand, J.P., Viallard, J.F., Jaccard, A., Hoarau, C., Lebranchu, Y., Bérezné, A., Mouthon, L., Karmochkine, M., Schleinitz, N., Durieu, I., Nove-Josserand, R., Chanet, V., Le-Moing, V., Just, N., Salanoubat, C., Jaussaud, R., Suarez, F., Hermine, O., Solal-Celigny, P., Hachulla, E., Sanhes, L., Gardembas, M., Pellier, I., Tisserant, P., Pavic, M., Bonnotte, B., Haroche, J., Amoura, Z., Alric, L., Thiercelin, M.F., Tetu, L., Adoue, D., Bordigoni, P., Perpoint, T., Sève, P., Rohrlich, P., Pasquali, J.L., Korganow, A.S., Soulas, P., Couderc, L.J., Catherinot, E., Giraud, P., Baruchel, A., Deleveau, I., Chaix, F., Donadieu, J., Tron, F., Larroche, C., Blanc, A.P., Masseau, A., Hamidou, M., Kanny, G., Morisset, M., Millot, F., Fain, O., Borie, R., Perlat, A., Bienvenue, B., Autran, B., Gorochov, G., Garnier, J.L., Moins, H., Maki, G., Fieschi, C., Malphettes, M., Boutboul, D., and Gérard, L. more...
- Abstract
In 2014, the European Society for Immune Deficiencies (ESID) revised the common variable immunodeficiency (CVID) diagnosis criteria by incorporating new clinical and biological markers. The new definition appeared more restrictive but had not yet been evaluated in a large cohort of patients. more...
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- 2016
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117. P31 Hémato - Oncologie Association piperacilline/tazobactam-netromycine dans le traitement des neutropenies febriles de l'enfant
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Pascal Chastagner, L. Mansuy, C. Schmitt, P. Bordigoni, F. Fouyssac, and Danièle Sommelet
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Pediatrics, Perinatology and Child Health - Published
- 2003
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118. Identification of New Prognosis Factors from the Clinical and Epidemiologic Analysis of a Registry of 229 Diamond-Blackfan Anemia Patients
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P. Bordigoni, G Tchernia, TN Willig, Stéphane Blanche, Annie Robert, J Budde, N Mohandas, Robert Girot, E Kohne, C. Tiemann, JL Stephan, S Eber, Niemeyer Cm, JM Guillard, A Lambiliotte, Guy Cornu, G. Souillet, and T Leblanc more...
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medicine.medical_specialty ,Pediatrics ,business.industry ,Anemia ,Incidence (epidemiology) ,Disease ,medicine.disease ,Short stature ,Premature birth ,Ribosomal protein S19 ,Pediatrics, Perinatology and Child Health ,Epidemiology ,Medicine ,medicine.symptom ,Diamond–Blackfan anemia ,business - Abstract
Diamond-Blackfan anemia (DBA) is a constitutional disease characterized by a specific maturation defect in cells of erythroid lineage. We have assembled a registry of 229 DBA patients, which includes 151 patients from France, 70 from Germany, and eight from other countries. Presence of malformations was significantly and independently associated with familial history of DBA, short stature at presentation (before any steroid therapy), and absence of hypotrophy at birth. Two hundred twenty-two patients were available for long-term follow-up analysis (median, 111.5 mo). Of these individuals, 62.6% initially responded to steroid therapy. Initial steroid responsiveness was found significantly and independently associated with older age at presentation, familial history of DBA, and a normal platelet count at the time of diagnosis. Severe evolution of the disease (transfusion dependence or death) was significantly and independently associated with a younger age at presentation and with a history of premature birth. In contrast, patients with a familial history of the disease experienced a better outcome. Outcome analysis revealed the benefit of reassessing steroid responsiveness during the course of the disease for initially nonresponsive patients. Bone marrow transplantation was successful in 11/13 cases; HLA typing of probands and siblings should be performed early if patients are transfusion dependent, and cord blood should be preserved. Incidence of DBA (assessed for France over a 13-y period) is 7.3 cases per million live births without effect of seasonality on incidence of the disease or on malformative status. Similarly, no parental imprinting effect or anticipation phenomenon could be documented in families with dominant inheritance. more...
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- 1999
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119. Anémie sévère chez un patient greffé renal: Infectionchronique à parvovirus B19
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J.L. André, J.C. Humbert, N. Martinet, J.F. Lesesve, P. Bordigoni, M.J. Krier, and R. Mareschal
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Pediatrics, Perinatology and Child Health - Published
- 1998
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120. Lymphohistiocytose familiale : étude rétrospective de 41 observations
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Hervé Rubie, P. Bordigoni, F. Mechinaud, J.-P. Vannier, Claire Galambrun, Gérard Michel, Alain Fischer, Jean-Louis Stephan, and N Philippe
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Pediatrics, Perinatology and Child Health - Published
- 1998
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121. Hepatite chronique c chez l'enfant: Epidemiologie et evolution (46 cas)
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A Morali, D Steschenko, P Bordigoni, P Chastagner, MJ Krier, JL André, A Lefacu, D Sommelet, and M Vidailhet
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Pediatrics, Perinatology and Child Health - Published
- 1996
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122. Allogeneic hematopoietic stem-cell transplantation for myeloid sarcoma: a retrospective study from the SFGM-TC.
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Chevallier P, Mohty M, Lioure B, Michel G, Contentin N, Deconinck E, Bordigoni P, Vernant J, Hunault M, Vigouroux S, Blaise D, Tabrizi R, Buzyn A, Socie G, Michallet M, Volteau C, and Harousseau J
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- 2008
123. Donor lymphocyte infusion in the treatment of first hematological relapse after allogeneic stem-cell transplantation in adults with acute myeloid leukemia: a retrospective risk factors analysis and comparison with other strategies by the EBMT Acute...
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Schmid C, Labopin M, Nagler A, Bornhäuser M, Finke J, Fassas A, Volin L, Gürman G, Maertens J, Bordigoni P, Holler E, Ehninger G, Polge E, Gorin NC, Kolb HJ, Rocha V, and EBMT Acute Leukemia Working Party more...
- Published
- 2007
124. GONADAL FUNCTIONS IN 24 PATIENTS AFTER BONE MARROW TRANSPLANTATION
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B LeHeud, M. Pierson, S Denet, D. Sommelet, and P. Bordigoni
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Pediatrics ,medicine.medical_specialty ,Chemotherapy ,Bone marrow transplantation ,business.industry ,medicine.medical_treatment ,hemic and immune systems ,chemical and pharmacologic phenomena ,Total body irradiation ,medicine.disease ,Surgery ,Lymphoma ,Regimen ,surgical procedures, operative ,Chronic leukemia ,immune system diseases ,hemic and lymphatic diseases ,Pediatrics, Perinatology and Child Health ,Medicine ,Ovarian dysfunction ,business ,Testicular dysfunction - Abstract
The long term follow-up of patients after bone marrow transplantation (BMT) allows for a more precise definition of its potential side-effects. Gonadal functions are potentially sensitive to several procedures regularly used for BMT preparations, either total body irradiation (TBI) or high dose regimen chemotherapy. We present a series of 24 patients aged at least 14 years previously treated by BMT, either before or after onset of puberty. There were 17 boys and 7 girls. Mean age at BMT was 13,2 for the boys and 11,2 tor the girls. 11 did not show any sign of pubertal development at the time of BMT. The indications for BMT were ALL in 11, ANLL in 2, lymphoma in 4, Chronic leukemia in 1, aplastic anaemia in 4 and solid tumors in 1. 13 received allogenic BMT and 11 autologous BMT. All the 7 girls, whatever the types of preparations and BMT, developed severe ovarian failure. Of the 17 boys, all 5 who have received previously testicular irradiation for ALL relapse and TBI before BMT have evidences of leydigian dysfunction (Δ). The evolution of the others 12 is summarized in the table according the status of puberty and conditioning regimens. Of the 9 given chemotherapy only, 3 were prepubere at BMT and 2 of these developed Sertolian dysfunction. In conclusion, this study confirms the constant ovarian dysfunction after BMT in girls. For the boys even in the group of patients given only chemotherapy there was evidence of secondary testicular dysfunction regardless of the respective timing ot BMT and the onset of puberty. more...
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- 1993
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125. Human Herpesvirus 6 Infection After Allogeneic Stem Cell Transplantation
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Jeulin, Helene, Agrinier, Nelly, Guery, Matthieu, Salmon, Alexandra, Clément, Laurence, Bordigoni, Pierre, and Venard, Véronique
- Abstract
Human herpesvirus 6 (HHV-6) is susceptible to latency and reactivation in hematopoietic stem cell transplant (HSCT) recipients. We investigated the incidence of HHV-6 DNAemia and factors related to HHV-6 DNAemia and death after allogeneic stem cell transplantations. We also explored the relationship between HHV-6 viral load and the presence of clinical signs. more...
- Published
- 2013
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126. Bone mineral density in adult survivors of childhood acute leukemia: impact of hematopoietic stem cell transplantation and other treatment modalities
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Le Meignen, Marion, Auquier, Pascal, Barlogis, Vincent, Sirvent, Nicolas, Contet, Audrey, Simeoni, Marie-Claude, Galambrun, Claire, Poirée, Marilyne, Chastagner, Pascal, Play, Barbara, Villes, Virginie, Berbis, Julie, Chambost, Hervé, Bordigoni, Pierre, and Michel, Gérard more...
- Abstract
Femoral and lumbar bone mineral densities (BMDs) were measured in 159 adults enrolled in the Leucémies de l'Enfant et de l'Adolescent program, a French prospective multicentric cohort of childhood leukemia survivors. BMDs were expressed as Z-scores, and multivariate linear regression analyses were used to construct association models with potential risk factors. Mean age at evaluation and follow-up was 23 and 14.7 years, respectively. In the whole cohort, mean femoral Z-score was −0.19 ± 0.08. Two factors were associated with lower femoral BMD transplantation (−0.49 ± 0.15 vs −0.04 ± 0.10 in the chemotherapy group; P= .006) and female sex (−0.34 ± 0.10 vs −0.03 ± 0.13; P= .03). Among patients who received a transplant, the only significant risk factor was hypogonadism (−0.88 ± 0.16 vs −0.10 ± 0.23; P= .04). A slight reduction in lumbar BMD (mean Z-score, −0.37 ± 0.08) was detected in the whole cohort without difference between the transplantation and chemotherapy groups. Among patients who received a transplant, younger age at transplantation was correlated with a low lumbar BMD (P= .03). We conclude that adults who had received only chemotherapy for childhood leukemia have a slight reduction in their lumbar BMD and a normal femoral BMD. Patients who received a transplant with gonadal deficiency have a reduced femoral BMD which might increase the fracture risk later in life. more...
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- 2011
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127. Prevalence and risk factors of the metabolic syndrome in adult survivors of childhood leukemia
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Oudin, Claire, Simeoni, Marie-Claude, Sirvent, Nicolas, Contet, Audrey, Begu-Le Coroller, Audrey, Bordigoni, Pierre, Curtillet, Catherine, Poirée, Maryline, Thuret, Isabelle, Play, Barbara, Massot, Mara Carazza, Chastagner, Pascal, Chambost, Hervé, Auquier, Pascal, and Michel, Gérard more...
- Abstract
We evaluate the prevalence and risk factors of the metabolic syndrome (MS) in young adults surviving childhood leukemia. During the years 2007 to 2008, assessment of MS was proposed to all adults included in the Leucémie de l'Enfant et de l'Adolescent program, a French prospective multicentric cohort of leukemia survivors. Among 220 eligible patients, 184 (83.6%) had complete evaluation. Median age at evaluation and follow-up duration were 21.2 and 15.4 years. Overall prevalence of MS was 9.2% (95% confidence interval, 5.5-14.4). There was no association of MS with sex, age at diagnosis, leukemia subtype, steroid therapy, and central nervous system irradiation. Patients were stratified according to 4 therapeutic modalities: chemotherapy alone (n = 97), chemotherapy and central nervous system irradiation (n = 27), hematopoietic stem cell transplantation (HSCT) without (n = 17) or with (n = 43) total body irradiation (TBI). MS occurred in 5.2%, 11.1%, 5.9%, and 18.6% of them, respectively. The higher risk observed in the HSCT-TBI group was significant in univariate and in multivariate analysis (odds ratio [OR] = 3.9, P = .03). HSCT with TBI was associated with a higher rate of hypertriglyceridemia (OR = 4.5, P = .004), low level of high-density lipoprotein cholesterol (OR = 2.5, P = .02), and elevated fasting glucose (OR = 6.1, P = .04) So, TBI is a major risk factor for MS. Further studies are warranted to explain this feature. more...
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- 2011
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128. Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)
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Schmid, Jana Pachlopnik, Canioni, Danielle, Moshous, Despina, Touzot, Fabien, Mahlaoui, Nizar, Hauck, Fabian, Kanegane, Hirokazu, Lopez-Granados, Eduardo, Mejstrikova, Ester, Pellier, Isabelle, Galicier, Lionel, Galambrun, Claire, Barlogis, Vincent, Bordigoni, Pierre, Fourmaintraux, Alain, Hamidou, Mohamed, Dabadie, Alain, Le Deist, Françoise, Haerynck, Filomeen, Ouachée-Chardin, Marie, Rohrlich, Pierre, Stephan, Jean-Louis, Lenoir, Christelle, Rigaud, Stéphanie, Lambert, Nathalie, Milili, Michèle, Schiff, Claudin, Chapel, Helen, Picard, Capucine, de Saint Basile, Geneviève, Blanche, Stéphane, Fischer, Alain, and Latour, Sylvain more...
- Abstract
X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP type 1 (XLP-1) is caused by mutations in the gene SH2D1A (also named SAP), whereas mutations in the gene XIAP underlie XLP type 2 (XLP-2). Here, a comparison of the clinical phenotypes associated with XLP-1 and XLP-2 was performed in cohorts of 33 and 30 patients, respectively. HLH (XLP-1, 55%; XLP-2, 76%) and hypogammaglobulinemia (XLP-1, 67%; XLP-2, 33%) occurred in both groups. Epstein-Barr virus infection in XLP-1 and XLP-2 was the common trigger of HLH (XLP-1, 92%; XLP-2, 83%). Survival rates and mean ages at the first HLH episode did not differ for both groups, but HLH was more severe with lethal outcome in XLP-1 (XLP-1, 61%; XLP-2, 23%). Although only XLP-1 patients developed lymphomas (30%), XLP-2 patients (17%) had chronic hemorrhagic colitis as documented by histopathology. Recurrent splenomegaly often associated with cytopenia and fever was preferentially observed in XLP-2 (XLP-1, 7%; XLP-2, 87%) and probably represents minimal forms of HLH as documented by histopathology. This first phenotypic comparison of XLP subtypes should help to improve the diagnosis and the care of patients with XLP conditions. more...
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- 2011
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129. Multicenter survey on the outcome of transplantation of hematopoietic cells in patients with the complete form of DiGeorge anomaly
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Janda, Ales, Sedlacek, Petr, Hönig, Manfred, Friedrich, Wilhelm, Champagne, Martin, Matsumoto, Tadashi, Fischer, Alain, Neven, Benedicte, Contet, Audrey, Bensoussan, Danielle, Bordigoni, Pierre, Loeb, David, Savage, William, Jabado, Nada, Bonilla, Francisco A., Slatter, Mary A., Davies, E. Graham, and Gennery, Andrew R. more...
- Abstract
Seventeen patients transplanted with hematopoietic cells to correct severe T lymphocyte immunodeficiency resulting from complete DiGeorge anomaly were identified worldwide, and retrospective data were obtained using a questionnaire-based survey. Patients were treated at a median age of 5 months (range, 2-53 months) between 1995 and 2006. Bone marrow was used in 11 procedures in 9 cases: 6 from matched unrelated donors, 4 from human leukocyte antigen (HLA)-identical siblings, and one haploidentical parent with T-cell depletion. Unmobilized peripheral blood was used in 8 cases: 5 from HLA-identical siblings, one from a matched unrelated donor, one from an HLA-identical parent, and one unrelated matched cord blood. Conditioning was used in 5 patients and graft-versus-host disease prophylaxis in 11 patients. Significant graft-versus-host disease occurred in 9 patients, becoming chronic in 3. Median length of follow-up was 13 months, with transplantation from HLA-matched sibling showing the best results. Median survival among deceased patients (10 patients) was 7 months after transplantation (range, 2-18 months). The overall survival rate was 41%, with a median follow-up of 5.8 years (range, 4-11.5 years). Among survivors, median CD3 and CD4 counts were 806 (range, 644-1224) and 348 (range, 225-782) cells/mm3, respectively, CD4+/CD45RA+cells remained very low, whereas mitogen responses were normalized. more...
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- 2010
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130. Comparison of In-House Real-Time Quantitative PCR to the Adenovirus R-Gene Kit for Determination of Adenovirus Load in Clinical Samples
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Jeulin, He´le`ne, Salmon, Alexandra, Bordigoni, Pierre, and Venard, Ve´ronique
- Abstract
ABSTRACTIn the context of hematopoietic stem cell transplantation, adenovirus infections are associated with relevant mortality and morbidity. Detection of adenovirus DNA by quantitative PCR is the “gold standard” for these patients. A total of 150 samples, namely, 78 whole-blood, 22 cerebrospinal fluid, 24 digestive biopsy, and 26 stool samples, from 29 patients, including 24 hematopoietic stem cell transplant recipients, were tested for the detection of adenovirus using an in-house real-time quantitative PCR assay (A. Heim, C. Ebnet, G. Harste, and P. Pring-Akerblom, J. Med. Virol. 70:228-239, 2003) and the commercially available Adenovirus R-Gene kit. Adenovirus DNA was automatically isolated from whole-blood samples (Magna Pure LC system; Roche) or was manually extracted from other specimens (QIAamp; Qiagen) using the appropriate kit. The intra- and interassay reproducibilities and sensitivities were evaluated with cell culture supernatant dilutions. Of the 150 samples tested, 86 were found to be positive and 55 were found to be negative using both techniques. Nine (6%) discordant results were obtained. In most cases, discrepant results concerned samples with low viral loads. Quantitative results for all concordant positive samples were analyzed using the Spearman correlation test. A good correlation between the results of the in-house assay and those of the kit assay was obtained (r= 0.95; P< 0.001). Regarding the threshold cycle value for internal control spiked samples, none of the 150 samples tested contained a PCR inhibitor. In conclusion, a relevant correlation of results between the in-house assay and the kit assay, as well as the high-quality reproducibility and sensitivity of the kit assay, warranted its use for follow-up of hematopoietic stem cell transplantation recipients. more...
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- 2010
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131. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease
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Bernaudin, Françoise, Socie, Gérard, Kuentz, Mathieu, Chevret, Sylvie, Duval, Michel, Bertrand, Yves, Vannier, Jean-Pierre, Yakouben, Karima, Thuret, Isabelle, Bordigoni, Pierre, Fischer, Alain, Lutz, Patrick, Stephan, Jean-Louis, Dhedin, Nathalie, Plouvier, Emmanuel, Margueritte, Geneviève, Bories, Dominique, Verlhac, Suzanne, Esperou, Hélène, Coic, Lena, Vernant, Jean-Paul, and Gluckman, Eliane more...
- Abstract
Allogeneic hematopoietic stem-cell transplantation (HSCT) is the only curative treatment for sickle cell disease (SCD); nevertheless, its use has been limited by the risk of transplantation-related mortality (TRM). Between November 1988 and December 2004, 87 consecutive patients with severe SCD ranging from 2 to 22 years of age received transplants in France. Cerebral vasculopathy was the principal indication for transplantation (55 patients). All the patients received grafts from a sibling donor after a myeloablative conditioning regimen (CR). The only change in the CR during the study period was the introduction of antithymocyte globulin (ATG) in March 1992. The rejection rate was 22.6% before the use of ATG but 3% thereafter. With a median follow-up of 6 years (range, 2.0 to 17.9 years), the overall and event-free survival (EFS) rates were 93.1% and 86.1%, respectively. Graft versus host disease (GVHD) was the main cause of TRM. Importantly, cord blood transplant recipients did not develop GVHD. No new ischemic lesions were detected after engraftment, and cerebral velocities were significantly reduced. The outcome improved significantly with time: the EFS rate among the 44 patients receiving transplants after January 2000 was 95.3%. These results indicate that HLA-identical sibling HSCT after myeloablative conditioning with ATG should be considered as a standard of care for SCD children who are at high risk for stroke. more...
- Published
- 2007
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132. Efficient generation of CD34+ progenitor‐derived dendritic cells from G‐CSF‐mobilized peripheral mononuclear cells does not require hematopoietic stem cell enrichment
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Paczesny, Sophie, Li, Yin‐Ping, Li, Na, Latger‐Cannard, Véronique, Marchal, Luc, Ou‐Yang, Jing‐Ping, Bordigoni, Pierre, Stoltz, Jean‐François, and Eljaafari, Assia
- Abstract
As a result of their potent antigen‐presentation function, dendritic cells (DC) are important tools for cell therapy programs. In vitro‐generated DC from enriched CD34+ hematopoietic stem cells (HSC; enriched CD34 DC) have already proven their efficiency in Phase I/II clinical trials. Here, we investigated whether enrichment of CD34+ HSC before the onset of culture was absolutely required for their differentiation into DC. With this aim, we developed a new two‐step culture method. PBMC harvested from G‐CSF‐mobilized, healthy patients were expanded for 7 days during the first step, with early acting cytokines, such as stem cell factor, fetal liver tyrosine kinase 3 ligand (Flt‐3L), and thrombopoietin. During the second step, expanded cells were then induced to differentiate into mature DC in the presence of GM‐CSF, Flt‐3L, and TNF‐α for 8 days, followed by LPS exposure for 2 additional days. Our results showed that the rate of CD34+/CD38+/lineagenegcells increased 19.5 ± 10‐fold (mean±sd) during the first step, and the expression of CD14, CD1a, CD86, CD80, and CD83 molecules was up‐regulated markedly following the second step. When compared with DC generated from enriched CD34+ cells, which were expanded for 7 days before differentiation, DC derived from nonenriched peripheral blood stem cells showed a similar phenotye but higher yields of production. Accordingly, the allogeneic stimulatory capacity of the two‐step‐cultured DC was as at least as efficient as that of enriched CD34 DC. In conclusion, we report herein a new two‐step culture method that leads to high yields of mature DC without any need of CD34+ HSC enrichment. more...
- Published
- 2007
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133. WHIM syndromes with different genetic anomalies are accounted for by impaired CXCR4 desensitization to CXCL12
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Balabanian, Karl, Lagane, Bernard, Pablos, José Luis, Laurent, Lysiane, Planchenault, Thierry, Verola, Olivier, Lebbe, Celeste, Kerob, Delphine, Dupuy, Alain, Hermine, Olivier, Nicolas, Jean-François, Latger-Cannard, Véronique, Bensoussan, Danièle, Bordigoni, Pierre, Baleux, Françoise, Le Deist, Françoise, Virelizier, Jean-Louis, Arenzana-Seisdedos, Fernando, and Bachelerie, Françoise more...
- Abstract
The WHIM syndrome is a rare immunodeficiency disorder characterized by warts, hypogammaglobulinemia, infections, and myelokathexis. Dominant heterozygous mutations of the gene encoding CXCR4, a G-protein–coupled receptor with a unique ligand, CXCL12, have been associated with this pathology. We studied patients belonging to 3 different pedigrees. Two siblings inherited a CXCR4 mutation encoding a novel C-terminally truncated receptor. Two unrelated patients were found to bear a wild-type CXCR4 open reading frame. Circulating lymphocytes and neutrophils from all patients displayed similar functional alterations of CXCR4-mediated responses featured by a marked enhancement of G-protein–dependent responses. This phenomenon relies on the refractoriness of CXCR4 to be both desensitized and internalized in response to CXCL12. Therefore, the aberrant dysfunction of the CXCR4-mediated signaling constitutes a common biologic trait of WHIM syndromes with different causative genetic anomalies. Responses to other chemokines, namely CCL4, CCL5, and CCL21, were preserved, suggesting that, in clinical forms associated with a wild-type CXCR4 open reading frame, the genetic anomaly might target an effector with some degree of selectivity for the CXCL12/CXCR4 axis. We propose that the sustained CXCR4 activity in patient cells accounts for the immune-hematologic clinical manifestations and the profusion of warts characteristic of the WHIM syndrome. more...
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- 2005
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134. Hematopoietic stem cell transplantation for complete IFN-γ receptor 1 deficiency: A multi-institutional survey
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Roesler, Joachim, Horwitz, Mitchell E., Picard, Capucine, Bordigoni, Pierre, Davies, Graham, Koscielniak, Ewa, Levin, Mike, Veys, Paul, Reuter, Ursula, Schulz, Ansgar, Thiede, Christian, Klingebiel, Thomas, Fischer, Alain, Holland, Steven M., Casanova, Jean-Laurent, and Friedrich, Wilhelm more...
- Abstract
To evaluate the outcome of hematopoietic stem cell transplantation (HSCT) in a series of patients with inherited complete IFN-γ receptor 1 (IFNγR1) deficiency.
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- 2004
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135. Comparative evaluation of schistocyte counting by an automated method and by microscopic determination.
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Lesesve, Jean-François, Salignac, Sylvain, Alla, François, Defente, Michael, Benbih, Mohamed, Bordigoni, Pierre, and Lecompte, Thomas
- Abstract
Schistocytes are circulating RBC fragments. The morphologic identification of schistocytes is difficult because the shapes to which they correspond are still under discussion. Automated hematology systems permit the possibility of direct measurement of RBC fragments. We compared schistocyte counts performed by different biologists and technicians with the automated counts by the ADVIA 120 (Bayer Health Care, Tarrytown, NY). The agreement between the ADVIA 120 and the average of the observers gives a correlation coefficient of 0.7274 (95% confidence interval, 0.6285-0.8019). The ADVIA 120 has a tendency to overestimate the count (average, +0.445%). No false-negative case was recorded. The maximum sensitivity (detection of 100% of samples with schistocytes) of the analyzer was determined at a threshold value of 0.25%, but the specificity was low (20%). Therefore, a blood smear examination remains necessary to confirm schistocyte presence. However, the clinical features correlated particularly with negative automated RBC fragments, and the high negative predictive value of RBC fragments ruled out thrombotic events (macroangiopathies or microangiopathies). more...
- Published
- 2004
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136. Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002
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Gennery, Andrew R., Khawaja, Khulood, Veys, Paul, Bredius, Robbert G. M., Notarangelo, Luigi D., Mazzolari, Evelina, Fischer, Alain, Landais, Paul, Cavazzana-Calvo, Marina, Friedrich, Wilhelm, Fasth, Anders, Wulffraat, Nico M., Matthes-Martin, Susanne, Bensoussan, Danielle, Bordigoni, Pierre, Lange, Andrzej, Pagliuca, Antonio, Andolina, Marino, Cant, Andrew J., and Davies, E. Graham more...
- Abstract
CD40 ligand (CD40L) deficiency causes recurrent sinopulmonary infection, Pneumocystis carinii pneumonia, and Cryptosporidium parvum infection. Approximately 40% to 50% of patients survive to the third decade: long-term survival is unclear. Hematopoietic stem cell transplantation (HSCT) is curative. We present a retrospective analysis of 38 European patients undergoing HSCT for CD40L deficiency in 8 European countries between 1993 and 2002. Donor stem cell source included 14 HLA-identical siblings, 22 unrelated donors, and 2 phenotypically matched parental stem cells (12 T-cell depleted). Of the patients, 34 engrafted and 26 (68%) survived; 3 had autologous reconstitution, 22 (58%) were cured, and 1 engrafted but has poor T-cell immune reconstitution. There were 18 evaluated patients who responded to vaccination. Of the patients, 12 (32%) died from infection-related complications, with severe cryptosporidiosis in 6. Grades 2 to 4 graft-versus-host disease (GvHD) associated with infection occurred in 6 of 12 fatal cases. HSCT cured 58% of patients, 72% of those without hepatic disease. Early T-cell function following whole marrow HSCT may limit cryptosporidial disease, but survival was similar after T-cell-depleted HSCT. Preexisting lung damage was the most important adverse risk factor. Further studies will determine optimal timing and type of HSCT. (Blood. 2004;103:1152-1157) more...
- Published
- 2004
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137. Unrelated cord blood transplantation for childhood acute myeloid leukemia: a Eurocord Group analysis
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Michel, Gerard, Rocha, Vanderson, Chevret, Sylvie, Arcese, William, Chan, Kah-Wah, Filipovich, Alexandra, Takahashi, Tsuneo A., Vowels, Marcus, Ortega, Juan, Bordigoni, Pierre, Shaw, Peter J., Yaniv, Isaac, Machado, Alexandra, Pimentel, Pedro, Fagioli, Franca, Verdeguer, Amparo, Jouet, Jean Pierre, Diez, Blanca, Ferreira, Euripedes, Pasquini, Ricardo, Rosenthal, Joseph, Sievers, Eric, Messina, Chiara, Iori, Anna Paola, Garnier, Federico, Ionescu, Irina, Locatelli, Franco, and Gluckman, Eliane more...
- Abstract
Results of unrelated cord blood transplantation (UCBT) in childhood acute myeloid leukemia (AML) have not been previously reported. We analyzed 95 children receiving UCB transplants for AML (20 in first complete remission [CR1], 47 in CR2, and 28 in more advanced stage). Poor prognosis cytogenetic abnormalities were identified in 29 cases. Most patients received a 1 or 2 HLA antigens-mismatched UCB transplants. The median number of collected nucleated cells (NCs) was 5.2 × 107/kg. Cumulative incidence (CI) of neutrophil recovery was 78% ± 4%, acute graft-versus-host disease (GVHD) was 35% ± 5%, and 100-day transplantation-related mortality (TRM) was 20% ± 4%. In multivariable analysis, a collected NC dose higher than 5.2 × 107/kg was associated with a lower 100-day TRM. The 2-year CI of relapse was 29% ± 5% and was associated with disease status. The 2-year leukemia-free survival (LFS) was 42% ± 5% (59% ± 11% in CR1, 50% ± 8% in CR2, and 21% ± 9% for children not in CR). Children with poor prognosis cytogenetic features had similar LFS compared with other patients (44% ± 11% vs 40% ± 8%). In CR2, LFS was not influenced by the length of CR1 (53% ± 11% in CR1 < 9.5 months compared with 50% ± 12% in later relapses). We conclude that UCBT is a therapeutic option for children with very poor-prognosis AML and who lack an HLA-identical sibling. (Blood. 2003;102:4290-4297) more...
- Published
- 2003
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138. Economic Evaluation of Recombinant Human Granulocyte Colony-Stimulating Factor in Very High-Risk Childhood Acute Lymphoblastic Leukemia
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Delorme, Jean, Badin, Stéphanie, Corroller, Anne-Gaelle G. Le, Auvrignon, Anne Aurtignon, Auclerc, Marie-Françoise, Gandemer, Virginie, Bordigoni, Pierre, Lamagnere, Jean-Pierre, Demeocq, François, Perel, Yves, Berthou, Christian, Bauduer, François, Pautard, Brigitte, Vannier, Jean-Pierre, Braguer, Diane, Leblanc, Thierry, Leverger, Guy, Baruchel, André, and Michel, Gérard more...
- Abstract
In a previous randomized study, the authors reported that granulocyte colony-stimulating factor (G-CSF) increased the chemotherapy dose-intensity delivered during the consolidation therapy of high-risk childhood acute lymphoblastic leukemia (ALL). The aim of the current study was to perform an economic evaluation in the same cohort. more...
- Published
- 2003
139. Evaluation of d-penicillamine in juvenile chronic arthritis. A double-blind, multicenter study
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P. Manigne, P. Bordigoni, C. Piussan, C. Griscelli, and A. M. Prieur
- Subjects
Male ,medicine.medical_specialty ,Adolescent ,Painful joints ,Immunology ,Anti-Inflammatory Agents ,Placebo ,Placebos ,Double blind ,chemistry.chemical_compound ,Double-Blind Method ,Rheumatology ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,Pharmacology (medical) ,Child ,Clinical Trials as Topic ,Nonsteroidal ,business.industry ,Penicillamine ,Juvenile chronic arthritis ,Arthritis, Juvenile ,Surgery ,Multicenter study ,chemistry ,Child, Preschool ,Joint pain ,Chronic Disease ,Prednisone ,Drug Therapy, Combination ,Female ,medicine.symptom ,business ,medicine.drug - Abstract
Seventy-four children with juvenile chronic arthritis were entered into a 6-month, multicenter, comparative double-blind study of the efficacy of D-penicillamine versus placebo. The results were evaluated in 70 patients, 55 of whom completed 6 months of the study. Improvement was observed in the total number of stiff joints, total number of painful joints, and total severity index measuring joint pain. There was also a significant reduction in the concurrent use of nonsteroidal anti-inflammatory drugs. D-penicillamine was well-tolerated in all but 2 patients. Some children in the placebo group exhibited definite improvement; however, relapses that were observed were mainly in that group. These results confirm the efficacy of D-penicillamine for the treatment of joint involvement in juvenile chronic arthritis. more...
- Published
- 1985
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140. Massive chemotherapy with autologous bone marrow transplantation in burkitt's lymphoma. A review of 50 patients treated in France
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Ross Pinkerton, Olivier Hartmann, Soulliet G, Laporte Jp, P. Bordigoni, Biron P, Philip T, J.L. Bernard, Freycon F, and Catherine Patte
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Chemotherapy ,Pathology ,medicine.medical_specialty ,Marrow transplantation ,business.industry ,medicine.medical_treatment ,Cell Separation ,Hematology ,General Medicine ,medicine.disease ,Autologous bone ,Burkitt Lymphoma ,Combined Modality Therapy ,Transplantation, Autologous ,Bleomycin ,Bone Marrow ,Antineoplastic Combined Chemotherapy Protocols ,medicine ,Drug Evaluation ,Humans ,business ,Burkitt's lymphoma ,Bone Marrow Transplantation ,Follow-Up Studies - Published
- 1985
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141. Treatment of chronic granulomatous disease with myeloablative conditioning and an unmodified hemopoietic allograft: a survey of the European experience, 1985-2000
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Seger, Reinhard A., Gungor, Tayfun, Belohradsky, Bernd H., Blanche, Stephane, Bordigoni, Pierre, Di Bartolomeo, Paolo, Flood, Terence, Landais, Paul, Müller, Susanna, Ozsahin, Hulya, Passwell, Justen H., Porta, Fulvio, Slavin, Shimon, Wulffraat, Nico, Zintl, Felix, Nagler, Arnon, Cant, Andrew, and Fischer, Alain more...
- Abstract
Treatment of chronic granulomatous disease (CGD) with myeloablative bone marrow transplantation is considered risky. This study investigated complications and survival according to different risk factors present at transplantation. The outcomes of 27 transplantations for CGD, from 1985 to 2000, reported to the European Bone Marrow Transplant Registry for primary immunodeficiencies were assessed. Most transplant recipients were children (n = 25), received a myeloablative busulphan-based regimen (n = 23), and had unmodified marrow allografts (n = 23) from human leukocyte antigen (HLA)–identical sibling donors (n = 25). After myeloablative conditioning, all patients fully engrafted with donor cells; after myelosuppressive regimens, 2 of 4 patients fully engrafted. Severe (grade 3 or 4) graft-versus-host disease (GVHD) disease developed in 4 patients: 3 of 9 with pre-existing overt infection, 1 of 2 with acute inflammatory disease. Exacerbation of infection during aplasia was observed in 3 patients; inflammatory flare at the infection site during neutrophil engraftment in 2: all 5 patients belonged to the subgroup of 9 with pre-existing infection. Overall survival was 23 of 27, with 22 of 23 cured of CGD (median follow-up, 2 years). Survival was especially good in patients without infection at the moment of transplantation (18 of 18). Pre-existing infections and inflammatory lesions have cleared in all survivors (except in one with autologous reconstitution). Myeloablative conditioning followed by transplantation of unmodified hemopoietic stem cells, if performed at the first signs of a severe course of the disease, is a valid therapeutic option for children with CGD having an HLA-identical donor. more...
- Published
- 2002
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142. Chronic graft-versus-host disease after allogeneic blood stem cell transplantation: long-term results of a randomized study
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Mohty, Mohamad, Kuentz, Mathieu, Michallet, Mauricette, Bourhis, Jean-Henri, Milpied, Noël, Sutton, Laurent, Jouet, Jean-Pierre, Attal, Michel, Bordigoni, Pierre, Cahn, Jean-Yves, Boiron, Jean-Michel, and Blaise, Didier more...
- Abstract
The use of peripheral blood stem cells (PBSCs) is rapidly growing in the allogeneic transplantation setting as an alternative to bone marrow (BM). We previously reported a higher incidence of chronic graft-versus-host disease (cGVHD) associated with allogeneic PBSC transplantation in a randomized trial. In this follow-up report, we analyzed the evolution of cGVHD in the patients (n = 101) enrolled on this study. At a median follow-up of 45 months (range, 31-57 months), we found that the 3-year cumulative incidence of cGVHD was 65% (95% confidence interval [CI] 51%-78%) in the PBSC group and 36% (95% CI 23%-49%) in the BM group (P= .004). We also found that extensive cGVHD was more frequent in the PBSC group (44% [95% CI 30%-58%] vs 17% [95% CI 7%-27%];P= .004). The prevalence of cGVHD was always higher in the PBSC arm. Ocular involvement was more frequent in PBSC recipients (P= .02). Cutaneous and liver involvement was similar among BM and PBSC recipients. Chronic GVHD required multiple courses of immunosuppressive therapy in addition to cyclosporine and corticosteroids during longer periods (P= .03). Altogether, this translated into longer periods of hospitalization after transplantation in the PBSC group (P= .04). Finally, we also confirm that cGVHD after PBSC transplantation is associated with an antileukemic effect that is at least as potent as after BM. However, to date, this has not translated into a survival difference, possibly due to the early-stage leukemic status of these patients or to the relatively small size of the study population. more...
- Published
- 2002
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143. Successful Extracorporeal Photochemotherapy for Chronic Graft-Versus-Host Disease in Pediatric Patients
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Halle, Pascale, Paillard, Catherine, D'Incan, Michel, Bordigoni, Pierre, Piguet, Christophe, De Lumley, Lionel, Stephan, Jean Louis, Berger, Marc, Rapatel, Chantal, Deméocq, François, and Kanold, Justyna more...
- Abstract
A total of 254 extracorporeal photochemotherapy (ECP) procedures were performed in 8 children (median age 10 years; range 5–15) with extensive resistant chronic graft-versus-host disease (GVHD). ECP was carried out in the pediatric environment using a Cobe Spectra separator and UV-MATIC irradiator. A peripheral venous with a single-lumen permanent central catheter access (69% of ECP-apheresis) or a dual-lumen permanent central catheter access (26% of ECP-apheresis) were used preferentially. A median platelet decrease of 17% (0–71) (p = 0.0001) and median hemoglobin level decrease of 15 g/L (0-31) (p = 0.0001) were noted following each ECP-apheresis. However, none of the patients had profound thrombocytopenia or anemia. Two minor episodes of catheter related-bacteriemia (Staphylococcus aureus) were noted (2310 catheter-days). A negative correlation was found between lymphocyte collection efficacy (median = 38%) and pre ECP-apheresis lymphocyte count (r = 0.4, p = 0.00001). The median of 5 × 107 lymphocytes/kg (0.1-50.107/kg) was irradiated in each procedure. All patients are alive and well, and 7/8 experienced a dramatic improvement in their cutaneous status. Liver and gut disease resolved completely in 4/6 and 5/5 patients, respectively. In all patients, a concomitant immunosuppressive therapy was stopped (5/8) or considerably reduced (3/8). Five patients with more than 2 years follow-up after discontinuation of ECP are in remission with no immunosuppression treatment. They have normal growth rates and normal school and sport activity. Our study shows that ECP is beneficial, well tolerated, and can be safely used for chronic GVHD treatment even in young children with low body weight and a poor performance status. We believe that having a dedicated pediatric environment together with an experienced, motivated, and specifically pediatric team is of crucial importance for improving patient's acceptance of this long-term therapeutic program. more...
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- 2002
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144. Quantitative Expression of Adhesion Molecules on Granulocyte Colony-Stimulating Factor-Mobilized Peripheral Blood, Bone Marrow, and Cord Blood CD34+ Cells
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Gigant, C., Latger-Cannard, V., Bensoussan, D., Feugier, P., Bordigoni, P., and Stoltz, J.F.
- Abstract
The purpose of this study is to investigate the function of the main adhesion receptors on CD34+ cells during hematopoietic stem cell transplantation. Expression was quantified by flow cytometry using calibration beads. CD34+ cells were isolated from either bone marrow (BM), cord blood (CB), or peripheral blood (PB) from study patients and a control group after granulocyte colony-stimulating factor (G-CSF) administration. The study of the CD34+ cell differentiation showed that CD34+ cells are mainly CD38+ and HLA-DR+, whatever the type of harvest. However, quantitative analysis elicited a weaker expression of CD38 on PB and CB CD34+ cells in comparison to BM CD34+ cells. The proportions of CD34+/CD49d+ and CD34+/CD49e+ were smaller on PB cells, without quantitative expression variation. This phenotypic variation promotes CD34+ cells to exit from BM into circulation, inducing the mobilization. The homing of the CD34+ cells to the BM involves the CD62L receptor. The expression of this receptor was found to be more frequent and stronger on PB cells than on BM or CB cells. The CD11b, CD18, and CD54 receptors are implicated in CD34+ cell adhesion to BM microenvironment. No significant variation in CD34+/CD11b+ and CD34+/CD18+ cell frequency was noted. Moreover, the CD54 receptor was more frequently expressed on CB and PB cells. Quantitative analysis revealed that CD18 was more strongly expressed on BM than on PB cells to promote progenitors adhesion by interacting with stromal cells. Finally, the quantitative expression of the main receptors on CD34+ cells explained cellular functions during the different steps of hematopoietic stem cells transplantation. more...
- Published
- 2001
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145. Investigation of aciclovir-resistant herpes simplex virus I infection in a bone marrow transplantation unit: genotyping shows that different strains are involved
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Venard, V., Dauendorffer, J.-N., Carret, A.-S., Corsaro, D., Edert, D., Bordigoni, P., and Faou, A.Le
- Abstract
Over an eight-month period from October 1997 to May 1998, four patients who had received a bone marrow transplant (BMT) from an unrelated donor presented with severe mucosal cutaneous infections involving aciclovir resistant herpes simplex virus 1 (HSV-1). The emergence within a short period of resistant HSV-1 strains in the bone marrow transplantation unit raised fears of hospital-acquired infections. The hypothesis was investigated by restriction fragment length polymorphism (RFLP), sequencing of the thymidine kinase (TK) gene and genotyping of hypervariable regions of these four strains. Restriction fragment length polymorphism proved to be poorly discriminant and the TK sequence did not rule out transmission between these patients. Amplification of reiterating hypervariable genomic HSV-1 regions designated Re IV and Re VII clearly differentiated patients' strains. Thus, in this study, there was no evidence of nosocomial transmission of HSV-1 strains between the four patients. more...
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- 2001
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146. Quantitative expression of adhesion molecules VLA-4, VLA-5, L-Selectin, Mac-1 and ICAM-1 on CD34+ cells
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Gigant, C., Latger-Cannard, V., Bensoussan, D., Feugier, P., Bordigoni, P., and Stoltz, J.
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- 2001
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147. Infection due to aciclovir resistant herpes simplex virus in patients undergoing allogeneic hematopoetic stem cell transplantation
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Venard, V., Dauendorffer, J. N., Carret, A. S., Corsaro, D., Edert, D., Bordigoni, P., and Faou, A. Le
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- 2001
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148. Long-term results of CD34<SUP>+</SUP> cell transplantation in children with neuroblastoma
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Kanold, Justyna, Yakouben, Karima, Tchirkov, Andrei, Carret, Anne-Sophie, Vannier, Jean-Pierre, LeGall, Edouard, Bordigoni, Pierre, and Deméocq, François
- Abstract
This is the first report of the long-term results of CD34+ cell transplantation in children with neuroblastoma. We investigated the hematologic and immune recovery, posttransplant morbidity, and clinical outcome of these children. Twenty-three children with advanced neuroblastoma had PBPCs (20 patients) or BM (3 patients) collected, followed by CD34+ cell selection on Ceprate column. The purge of residual neuroblastoma cells was evaluated using an RT-PCR for tyrosine hydroxylase (TH) mRNA assay. Reinfusion of CD34+ cells followed busulfan + melphalan myeloablative chemotherapy. A median of 2.9 × 106 CD34+ cells/kg was reinjected. Median days to achieve ANC > 0.5 × 109/liter and platelets > 50 × 109/liter were 13 (range 933) and 59 (range 22259), respectively. Circulating T cells were primarily CD4−/CD8+ with fewer than 0.2 109CD4+ cells/liter throughout the first 6 months. CD19+ cells and CD56+ cells were not detectable up to day +35 posttransplant. At 1 year posttransplant, 16 evaluable patients had stable hematopoiesis with 2.3 × 109 ANC/liter (range 0.84.1), 1.4 × 109 lymphocytes/liter (range 0.5 2.0) and 251 × 109 PLT/liter (range 35490). After the completion of hematopoietic reconstitution, six events of severe septicemia/septic shock were noted. Six children had severe VZV infections, and 2 had EBV-associated lymphoproliferation. Thirteen patients are alive with a median follow-up of 40 months (range 254). Ten patients have died; 8 relapsed or developed progressive disease, 1 died from nondocumented pneumopathy at day 56, and 1 developed AML-M4 at 3 years posttransplant. In children, CD34+ cell transplantation can be accomplished with a reduction of neuroblastoma cell inoculum in the selected graft as assessed by RT-PCR analysis. CD34+ cell grafts provide successful neutrophil reconstitution. However, delayed platelet recovery, persistent decrease in CD4+ lymphocyte levels and a high incidence of serious and life-threatening late infections were observed in these children. There remains a critical need to evaluate any real clinical benefit of CD34+ cell autografts in neuroblastoma patients. Med. Pediatr. Oncol. 35:17, 2000. © 2000 Wiley-Liss, Inc. more...
- Published
- 2000
- Full Text
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149. Long-term effect of bone-marrow transplantation for childhood-onset cerebral X-linked adrenoleukodystrophy
- Author
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Shapiro, E, Krivit, W, Lockman, L, Jambaqué, I, Peters, C, Cowan, M, Harris, R, Blanche, S, Bordigoni, P, Loes, D, Ziegler, R, Crittenden, M, Ris, D, Berg, B, Cox, C, Moser, H, Fischer, A, and Aubourg, P more...
- Published
- 2000
- Full Text
- View/download PDF
150. Genotyping of adenoviruses isolated in an outbreak in a bone marrow transplant unit shows that diverse strains are involved
- Author
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Venard, V., Carret, A.-S., Corsaro, D., Bordigoni, P., and Faou, A.Le
- Abstract
In the Bone Marrow Transplant Unit of the “Centre Hospitalier Universitaire” in Nancy, from October 1995 to May 1996, 13 patients of 65 (20%) had a positive adenovirus (Ad) culture after bone marrow transplant. This unusually high rate raised fears of nosocomial spread and so isolates were serotyped. Fourteen Ad strains were isolated from the 13 patients, nine were of serotype 1, 2 or 3, and 5 were non-typable. These five latter strains were responsible for four cases of severe infection with fatal outcome within a two-month period. They were further submitted to restriction fragment length polymorphism analysis of their DNA which showed the isolates differed by a percentage similarity of 8–79%. In this outbreak, different strains were involved, and there was no evidence of a nosocomial origin of Ad infection. more...
- Published
- 2000
- Full Text
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