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Introduction: Development of additional parameters for complete blood count has emerged in recent hematology analyzers, leading to many publications. However, few studies have been conducted on advanced RBC parameters and hemolytic anemias. We investigated the interest of Sysmex unique parameters, MicroR and HypoHe, as well as the immature fraction of reticulocytes (IRF) in combination with complete blood and reticulocyte count, for screening hereditary spherocytosis (HS) and pyruvate kinase deficiency. Methods: We analyzed 182 samples using Sysmex XE‐5000 analyzers from a cohort of red cell disorder patients from the Rouen University Hospital. These included 47 HS, 17 pyruvate kinase deficiencies, sickle cell diseases and trait, β‐thalassemia minor, iron deficiencies, and 489 samples from a routine group. Results: Combining five parameters (hemoglobin level, reticulocyte count, IRF, MicroR, and %HypoHe), we developed a specific screening tool for HS allowing a sensitivity of 100% and a specificity of 92.1% and a specific screening tool for pyruvate kinase deficiencies allowing a sensitivity of 100% and a specificity of 96.5%. These parameters were also found accurate in infants and in HS without anemia. Conclusion: We propose a costless, easy‐to‐use, and efficient approach to detect HS and pyruvate kinase deficiencies using Sysmex analyzers. These screening tools may help diagnosis of these disorders, help prevent complications, and result in a better management of these patients. [ABSTRACT FROM AUTHOR]

The paper presents an overview of the various types of libraries in France, including public libraries, academic and research libraries, other types of libraries, documentation, France’s library policy and the library staff and professional association environment The paper is based on a chapter from Les bibliothèques en Europe, projets,perspectives, (Libraries in Europe, Projects and Prospects) by Frederic Blin and Thierry Claerr, Paris, Editions du Cercle de la Librairie, 2013, with kind permission of the publisher and authors. [ABSTRACT FROM PUBLISHER]

Copyright of Bulletin des bibliothèques de France is the property of ENSSIB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Bulletin des bibliothèques de France is the property of ENSSIB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Bulletin des bibliothèques de France is the property of ENSSIB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Bulletin des bibliothèques de France is the property of ENSSIB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Bulletin des bibliothèques de France is the property of ENSSIB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The author suggests that public libraries in France may not be impacted by the French government's third decentralization phase (Acte III de la décentralisation) and discusses a proposal by the Interassociation Archives-Bibliothèques-Documentation (IABD) to review the Code du patrimoine (heritage code).

We report two members of a French family who are carriers of a rare hemoglobin (Hb) variant leading to erythrocytosis: Hb Saint Nazaire [β103(G5)Phe→Ile; HBB : c.310T>A]. The proband is a 38-year-old woman referred to our institution for a moderate but persistent polycythemia without any clinical consequence. As her mother had a similar blood count, a diagnosis of a Hb variant with high oxygen affinity was proposed. The variant was difficult to detect by capillary electrophoresis (CE) and not distinguishable by high performance liquid chromatography (HPLC) and isoelectric focusing. Finally, a heterozygous mutation on the HBB gene corresponding to Hb Saint Nazaire was identified. This case report illustrates that this rare cause of erythrocytosis can be easily under or misdiagnosed unless several Hb separation techniques are used.

Objectives: To analyze the main characteristics of adults with sickle cell disease (SCD) and concurrent connective tissue disease (CTD)., Methods: A retrospective investigational study was performed. CTD was diagnosed according to standard international criteria. Severity of SCD was assessed by a clinical severity score., Results: Thirty patients, 23 women (76%) and 7 men, with hemoglobin S/S (n = 25) or S/C (n = 5) SCD were included. The subtypes of CTD were rheumatoid arthritis (RA) (n = 15), definite systemic lupus erythematosus or "incomplete lupus" requiring treatment (n = 13), primary Sjögren's syndrome with central nervous system involvement (n = 1), and systemic sclerosis (n = 1). Twenty-five of the 30 patients (83%) received steroid treatment, and 15 (50%) received at least 1 immunosuppressive agent (methotrexate in 14 cases) to control CTD. Four RA patients were given antitumor necrosis factor (TNF)alpha and 1 was treated with rituximab without SCD exacerbation. After a median follow-up of 4.5 years [range: 6 months to 30 years] from CTD diagnosis, 11 of the 25 (44%) patients receiving steroids had at least 1 episode of severe infection (mostly due to Staphylococcus aureus or Escherichia coli). SCD exacerbated in 13 of the 30 (43%) patients after CTD onset; 12 of these patients were receiving prednisone and/or methotrexate. Six patients (20%) had died from sepsis (n = 2), stroke (n = 2), or acute chest syndrome (n = 2)., Conclusions: CTD-related clinical manifestations and outcome were not particularly severe in patients with SCD. However, those with active CTD and undergoing steroid +/- methotrexate treatment had more serious SCD-related manifestations, a higher rate of severe infections, and an overall patient mortality rate of 20%. Thus, the management of patients with CTD and underlying SCD should consider the risk/benefit ratio of each treatment and steroid-sparing strategies should be implemented.