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Tulliez M.,Groupe Hospitalier University Henri Mondor
Revue Francophone des Laboratoires | Year: 2011

Chronic myeloid leukaemia is a model of how the molecular understanding of a disease can transform the therapy and the monitoring. imatinib which targets the oncogene product BCR ABL has transformed the natural history of this disease during the last decade. The recognition that some patients develop mutations within BCR-ABL induced the development of the second generation tyrosine kinase inhibitors (TKI), nilotinib and dasatinib. Both compounds are more potent than imatinib and have prooved effective in patients with CML in failure with imatinib. T315I mutation on BCR-ABL confers resistance to all three drugs. Third generation of TKI is now appearing, targeting this highly resistant mutation. Imatinib can be safely discontinued in patients with complete molecular remission of at least two years duration; a longer follow up is needed but the 40% of patients who had not relapsed are highly promising. Within the next years a cure of patients may been considered if two conditions are fullfilled:make the treatment discontinuation available for all patients: nilotinib and dasatinib for newly diagnosed chronic myeloid leukemia are superior to imatinib with higher rates of complete cytogenetic remission, faster time to remission, and reduced rates of progression to accelerated phase or blast crisis. The addition of Peg Interferon α2A to imatinib therapy in first line, results in significantly higher rates of molecular response;decrease the probability of relapse after treatment discontinuation with strategies targeting the leukemic stem cell.

Tran Houangkeo T.H.Y.,Center Hospitalier University Pitie Salpetriere | Bodereau V.,Groupe Hospitalier University Henri Mondor | Riou J.,Groupe Hospitalier University Henri Mondor | Pissard S.,Groupe Hospitalier University Henri Mondor | Pissard S.,University Paris Est Creteil
Hemoglobin | Year: 2016

Hb Savaria [α49(CE7)Ser→Arg; HBA2: c.150C > A] is a rare hemoglobin (Hb) variant, initially described in Eastern Europe but present worldwide. It belongs to that class of variants which can be confused with Hb S [β6(A3)Glu→Val; HBB: c.20A > T] by automated protein analysis and thus needs special tests for proper identification. Because it could arise from different nucleotide substitutions and according to the rules of the Human Genome Variation Society (HGVS) nomenclature, three ‘Hb Savaria’ variants are possible. In the case reported here it resulted from the HBA2: c.148A > C change. © 2016 Informa UK Limited, trading as Taylor & Francis Group.

Richard J.-C.M.,Charles University | Richard J.-C.M.,Institute for Biomedical Research | Richard J.-C.M.,University of Geneva | Pham T.,Groupe Hospitalier University Henri Mondor | And 15 more authors.
Critical Care | Year: 2012

Introduction: The specific burden imposed on Intensive Care Units (ICUs) during the A/H1N1 influenza 2009 pandemic has been poorly explored. An on-line screening registry allowed a daily report of ICU beds occupancy rate by flu infected patients (Flu-OR) admitted in French ICUs.Methods: We conducted a prospective inception cohort study with results of an on-line screening registry designed for daily assessment of ICU burden.Results: Among the 108 centers participating to the French H1N1 research network on mechanical ventilation (REVA) - French Society of Intensive Care (SRLF) registry, 69 ICUs belonging to seven large geographical areas voluntarily participated in a website screening-registry. The aim was to daily assess the ICU beds occupancy rate by influenza-infected and non-infected patients for at least three weeks. Three hundred ninety-one critically ill infected patients were enrolled in the cohort, representing a subset of 35% of the whole French 2009 pandemic cohort; 73% were mechanically ventilated, 13% required extra corporal membrane oxygenation (ECMO) and 22% died. The global Flu-OR in these ICUs was only 7.6%, but it exceeded a predefined 15% critical threshold in 32 ICUs for a total of 103 weeks. Flu-ORs were significantly higher in University than in non-University hospitals. The peak ICU burden was poorly predicted by observations obtained at the level of large geographical areas.Conclusions: The peak Flu-OR during the pandemic significantly exceeded a 15% critical threshold in almost half of the ICUs, with an uneven distribution with time, geographical areas and between University and non-University hospitals. An on-line assessment of Flu-OR via a simple dedicated registry may contribute to better match resources and needs. © 2012 Richard et al.; licensee BioMed Central Ltd.

Meziere A.,Charles University | Meziere A.,Groupe Hospitalier University Henri Mondor | Audureau E.,Groupe Hospitalier University Henri Mondor | Audureau E.,University Paris Est Creteil | And 7 more authors.
Journals of Gerontology - Series A Biological Sciences and Medical Sciences | Year: 2014

Background: Cobalamin deficiency is responsible for hematological, neurological, neurocognitive, and neuropsychiatric impairments and is a risk factor for cardiovascular diseases, particularly in the elderly people. Methods: In order to determine B12 status in old inpatients, a total number of 14,904 hospitalized patients in whom B12 measurements were performed in five hospitals in the Paris metropolitan area were included from January 1, 2011 to December 31, 2011. The aims of the study were to determine whether age had an impact on B12 and folate deficiencies and to evaluate correlations between B12 and biological parameters-folate, hemoglobin, mean cell volume, homocystein (tHcy)- and age. Results: Patients were aged 70.3 ± 19.5 years. Low B12 concentration (<200 ng/L) was observed in 4.6% of cases, 24.2% had middle B12 concentration (200-350 ng/L), 12.6% were functional B12 deficient (B12 < 350 ng/L associated to high tHcy level, tHcy > 17 μmol/L), 20.4% had low folate concentration (folate < 4 μg/L), 10.6% were functional folate deficient (folate < 4 μg/L associated to tHcy > 17 μmol/L), and 4.7% of patients were both functional B12 and folate deficient. The B12 or folate deficient patients had lower mean cell volume level than nondeficient patients. Increase in mean cell volume and tHcy concentrations with age and decrease in B12, folate, and hemoglobin levels with age were observed. Frequency of functional B12 deficiency was 9.6% in patients aged 30-60 years and 14.2% in patients over 90 years. Frequency of functional folate deficiency was 9.5% in 30-60 years and 12.1% in >90 years. Conclusions: In inpatients, functional B12 deficiency and functional folate deficiency increase with age and are not associated with anemia or macrocytosis. False vitamin B deficiencies are frequent. © The Author 2014. Published by Oxford University Press on behalf of The Gerontological Society of America.

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