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To determine whether a risk factor adapted chemotherapy would improve the outcome of non-metastatic bone Ewing's sarcoma.Standard risk tumours (SR, good histological response to chemotherapy or small unresected tumours) received the previous EW88 chemotherapy. Ifosfamide/etoposide (IE) were introduced after 3 courses of cyclophosphamide/doxorubicine when tumour regression was50% or during consolidation therapy for the intermediate risk tumours (IR, intermediate histological response 5-30% residual cells or large unresected tumours100ml). High risk tumours (HR, histological poor response30% residual cells or clinical poor response50% for unresectable tumours), received IE prior high dose busulfan/melphalan with stem cell rescue.From 1993 to 1999, 214 patients were enrolled. 5 y-EFS and OS were 60% (95% confidence interval (CI), 53-66) and 69% (95% CI, 63-75), respectively. 116 (54%), 46 (21%), 48 (22%) patients were considered as SR, IR and HR of relapse, respectively. No advantage to IE was observed in the IR group. As compared to previous study, tumour with poor histological response to induction chemotherapy seemed to benefit from the consolidation strategy including busulfan/melphalan: EFS were 45% (95% CI, 30-60) and 20% (95% CI, 7-43) for EW93 and EW88, respectively. Despite a risk-adapted strategy, histological response to chemotherapy remains the main prognostic factor in resected tumours, while initial tumour volume is the main prognostic factor for unresected tumours.These results showing a potential benefit of a consolidation strategy including busulfan/melphalan as compared to conventional chemotherapy needed confirmation by a randomised trial and were one of the bases of the ongoing EuroEwing99.

Aim of the study To evaluate the prognostic significance of the initial cerebro-spinal fluid (CSF) involvement of children with ALL enrolled from 1989 to 1996 in the EORTC 58881 trial. Patients and methods Patients (2025) were categorised according to initial central nervous system (CNS) status: CNS-1 (CNS negative, n = 1866), CNS-2 ( 3 , CSF with blasts, n = 50), CNS-3 (CNS positive, n = 49), TLP+ (TLP with blasts, n = 60). CNS-directed therapy consisted in intravenous (i.v.) methotrexate (5 g/sqm) in 4–10 courses, and intrathecal methotrexate injections (10–20), according to CNS status. Cranial irradiation was omitted in all patients. Results In the CNS1, TLP+, CNS2 and CNS3 group the 8-year EFS rate (SE%) was 69.7% (1.1%), 68.8% (6.2%), 71.3% (6.5%) and 68.3% (6.2%), respectively. The 8-year incidence of isolated CNS relapse (SE%) was 3.4% (0.4%), 1.7% (1.7%), 6.1% (3.5%) and 9.4% (4.5%), respectively, whereas the 8-year isolated or combined CNS relapse incidence was 7.6% (0.6%), 3.5% (2.4%), 10.2% (4.4%) and 11.7% (5.0%), respectively. Patients with CSF blasts had a higher rate of initial bad risk features. Multivariate analysis indicated that presence of blasts in the CSF had no prognostic value: (i) for EFS and OS; (ii) for isolated and isolated or combined CNS relapse; WBC count 9 /L and Medac E-coli asparaginase treatment were each related to a lower CNS relapse risk. Conclusions The presence of initial CNS involvement has no prognostic significance in EORTC 58881. Intensification of CNS-directed chemotherapy, without CNS radiation, is an effective treatment of initial meningeal leukaemic involvement.

To translate, cross-culturally adapt, and validate the functional disability tool Childhood Health Assessment Questionnaire (CHAQ), a variant of the Health Assessment Questionnaire (HAQ), in children with juvenile idiopathic arthritis (JIA).The disability index is the mean of the scores on the eight domains of the CHAQ and can range from 0 (no disability) to 3 (maximum disability). The CHAQ was first translated into French and adapted, then validated in a multicenter cross-sectional study in 306 children with JIA (systemic onset, 23%; polyarticular onset, 22%; extended oligoarticular subtype, 25%; and persistent oligoarticular subtype, 30%).Overall CHAQ scores discriminated between the four JIA subtypes (systemic: 1.1 +/- 0.9; polyarticular: 0.8 +/- 0.7, extended oligoarticular 0.8 +/- 0.7, and persistent oligoarticular: 0.4 +/- 0.5 [P0.0001]). Reproducibility evaluated by test-retest at a 7-day interval was excellent (intraclass coefficient, 0.91), as was agreement between the Parent's and Children's versions of the questionnaire (intraclass coefficient, 0.89). Significant correlations were found between the overall CHAO score and variables reflecting disease severity (joint counts, physician's and parent's global assessments, and erythrocyte sedimentation rate), indicating excellent convergent validity of the tool.The French version of the CHAQ displays good psychometric characteristics, although its sensitivity to change remains to be established. The French version of the CHAO should prove useful in international studies and can be expected to be helpful for monitoring individual patients with JIA.

Resume Objectifs. Traduire, adapter au contexte culturel francais, et valider chez les enfants souffrant d’Arthrite Juvenile Idiopathique (AJI) un instrument d’incapacite fonctionnelle, le Childhood Health Assessment Questionnaire (CHAQ), adaptation du Health Assessment Questionnaire (HAQ). Enfants et methodes. Apres avoir produit une traduction-adaptation francaise du CHAQ, l’instrument a ete valide lors d’une etude multicentrique transversale chez 306 enfants souffrant d’AJI (forme systemique 23 %, forme polyarticulaire 22 %, forme oligoarticulaire etendue 25 %, forme oligoarticulaire persistante 30 %). Resultats. Le score global du CHAQ correspond a la moyenne des scores obtenus pour chacun des huit domaines d’activite explores par l’instrument et varie entre 0 (absence d’incapacite fonctionnelle) et trois (incapacite fonctionnelle maximale). Le score global du CHAQ differe significativement entre les quatre types d’AJI (formes systemiques : 1,1 ± 0,9, formes polyarticulaires : 0,8 ± 0,7, formes oligoarticulaires etendues : 0,8 ± 0,7, formes oligoarticulaires persistantes : 0,4 ± 0,5 〚p

Extranodal thyroid lymphomatous involvement is rare in childhood. We report here 2 children, 1 with vertical transmission-acquired human immunodeficiency virus (HIV), presenting with lymphomatous infiltration of the thyroid gland at diagnosis. One child had infra-clinical endocrine impairment and both responded well to chemotherapy. Although the cases are too scarce to be affirmative, thyroid gland involvement doesn't seem to alter the good prognosis of childhood Burkitt's lymphoma. The third child's cancer in frequency is Non-Hodgkin Lymphomas. Presenting as the initial AIDS event in 1 patient, this case report also highlights the need to systematically propose antiretroviral therapy in vertically HIV infected children.

Umbilical cord blood can now be considered as an effective source of cells for hematopoietic reconstitution in HLA-identical situations. But it also seems important to evaluate the immune capacity of these cells as they need to have an antileukemic effect when transplanted for acute leukemia. We analyzed 51 umbilical cord blood samples from normal newborns (37–41 weeks old). Phenotypic analysis of cells with monoclonal antibodies and with the use of flow cytometry showed relative values of CD2, CD3, and CD8, identical to normal adult peripheral blood controls (CD2: 78.6 ± 10%; CD3: 70 ± 13.1%, CD8: 22.1 ± 6.1%). We noted higher values of CD4+ cells: 52.7 ± 9% ( P

Traitement du syndrome d’hemophagocytose dans l’intolerance aux proteines dibasiques avec lysinurie (IPDL). Introduction L’IPDL est une anomalie hereditaire rare du metabolisme des acides amines dibasiques. Cliniquement, la maladie se manifeste par des symptomes digestifs avec hepatosplenomegalie et retard de croissance. Biologiquement, le diagnostic repose sur une hyperammoniemie avec hyperaminoacidurie dibasique. L’atteinte hematologique peut comporter une cytopenie peripherique principalement par hypersplenisme et les signes biologiques du syndrome d’activation macrophagique. Le mye-logramme peut etre normal ou retrouver une phagocytose particuliere des erythroblasts et des polynucleaires neutrophiles simultanement par des macrophages et des progeniteurs granuleux, specifique de l’IPDL. Cas clinique Il s’agit d’un garcon de 13 ans, dont le diagnostic d’IPDL a ete pose a l’âge de 15 mois sur une volumineuse hepatosplenomegalie avec symptomatologie digestive pour laquelle une nutrition parenterale pauvre en protides a du etre introduite. L’evolution de sa maladie a ete marquee par de multiples episodes de septicemie sur catheter central au cours desquelles l’enfant presentait a chaque fois des pancytopenies mises sur le compte de l’hypersplenisme, spontanement resolutives donc non explorees. Lors de sa derniere septicemie, une hemophagocytose biologique et medullaire specifique telle qu’elle est decrite dans l’IPDL a ete mise en evidence. Cet enfant a ete traite par des corticoides IV 5 jours a 2 mg/kg/j puis per os en decroissance sur 15 jours, associes a de la ciclosporine IV 7 jours a 2,5 mg/kg/j puis per os a 6 mg/kg/j depuis 3 mois toujours en cours de decroissance. L’evolution des signes biologiques de l’hemophagocytose a ete marquee par une normalisation initiale pendant les 20 premiers jours de traitement puis une rechute a l’arret des corticoides. Conclusion Un myelogramme doit etre effectue devant toute pan-cytopenie chez un patient atteint d’une IPDL a la recherche de signes specifiques d’hemophagocytose. Le traitement par ciclosporine et corticoides semble permettre une normalisation des signes biologique de l’hemophagocytose plus rapidement qu’en l’absence de traitement et semble donc interessante surtout dans des contextes infectieux.

Department of Paediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France Department of Biostatistics, Institut Gustave Roussy, Villejuif, France Department of Paediatric Oncology, Institut d’Hemato-Oncologie Pediatrique, Lyon, France Department of Paediatric Oncology, Institut Curie, Paris, France Department of Paediatric Oncology, Hopital La Timone, Marseille, France Department of Paediatric Hematology-Oncology, Hopital Trousseau, Paris, France Department of Medical Oncology, Centre Claudius Regaud, Toulouse, France Department of Paediatric Oncology, Centre Oscar Lambret, Lille, France Department of Paediatric Hematology Oncology, Hopital Clocheville, Tours, France Department of Paediatric Hematology Oncology, Centre Hospitalie Regional de Besanc on, Besanc on, France Department of Paediatric Hematology Oncology, Hopital d’enfants, Nancy, France Department of Medical Oncology, Institut Bergonnie, Bordeau, France Department of Paediatric Hematology Oncology, Centre Hospitalie Universitaire, Caen, France Department of Paediatric Hematology Oncology, Centre Hospitalie Universitaire, Rennes, France Department of Paediatric Hematology Oncology, Hopiyal americain, Reins, France Department of Paediatric Hematology Oncology, Hopital Charles Nicolle, Rouen, France Department of Paediatric Hematology Oncology, CHU A. Michalon, Grenoble, France Department of Paediatric Hematology Oncology, Hopital Hautepierre, Strasbourg, France