Background. - Serum protein electrophoresis (SPE) and immunofixation electrophoresis (IFE) are used for diagnosis and follow-up of patients with intact immunoglobulin multiple myeloma. However, the numerous limitations of these methods led to the development of a nephelometric immunoassay (Hevylite (TM)) for the specific measurement of serum IgG kappa, IgG lambda, IgA kappa and IgA lambda concentrations.Methods. - In this study, we evaluated the correlation between this assay and SPE and IFE in 114 sera of 15 patients (12 IgG and 3 IgA patients) and its impact on the clinical care of patients, especially for diagnosis, for the evaluation of residual disease and for early detection of relapse.Results. - At inclusion and during follow-up, we found a good correlation between monoclonal immunoglobulin concentrations and SPE (R-2 = 0.902 for IgA and R-2 = 0.915 for IgG) and nephelometric quantification (R-2 = 0.948 for IgA and R-2 = 0.920 for IgG) for the evaluation of monoclonal and polyclonal immunoglobulins. Our results illustrate that the Hevylite (TM) test is less sensitive than the IFE for detection of residual disease: 5 patients who obtained very good partial response or complete response had normalization of the Hevylite (TM) ratio while IFE was still positive. A relapse had been detectable with the Hevylite (TM) ratio I to 2 months earlier than with SPE and IFE in 3 patients out of 15, but no recommendations for treating patients with only slight biological relapse are available.Conclusion. - Our results demonstrate that heavy/light chain specific immunoglobulin ratios provides no additional information than serum proteins electrophoresis and immunofixation for the diagnosis and the follow-up of intact immunoglobulin multiple myeloma patients. We also studied the correlation between the concentration of total immunoglobulin measured by Hevylite (TM) (sum of Ig'kappa + Ig'lambda) and nephelometric measurement of total IgG or IgA. For this correlation analysis, all 114 sera were analyzed. The correlation coefficient was R-2 = 0.948 for IgA and R-2 = 0.920 for IgG. (C) 2015 Elsevier Masson SAS. All rights reserved.

smoldering MM (SMM). Samples from patients with MGUS and healthy donors were also included in the study. LEN treatment was performed at concentration ranging from 0,1 to 2 M during all the differentiation culture period. Moreover moDC differentiation was performed in the presence of CM of human TERT transfected-hMSCs (hTERT-hMSCs) treated for 5 days with LEN. DC maturation markers (CD83, HLA-DR, CD80, CD86 and CD209) were evaluated by flow cytometry. The levels of soluble factors produced by mo-DCs were measured in the conditioned media (CM) of mo-DC by a Bio-Plex Multiplex System. The expression levels of Ikaros and Aiolos were also evaluated by western blot. LEN treatment induced a reduction of both number and % of mature mo-DC as compared to untreated controls. On the other hand, LEN treatment significantly increased the median intensity expression of CD86 HLA-DR and CD209 but not of CD80 by moDCs derived from BM. Similarly increased CD209 expression has been also seen in mo-DCs derived from PB in both MM and SMM patients. LEN treatment enhanced the production of IL-8 and MCP1 and decreased TNFlevels. Ikaros mediated the effect of LEN on DCs as we observed a significant down-regulation of Ikaros levels in THP1-DCs after LEN treatment with a dose dependent effect. On the other hand, we found that LEN blunted the inhibitory effect of hTERT-hMSCs on mo-DC differentiation in three independent experiments and slightly down-regulated IL-6 and IDO but not TGFB1, IL-8, TNF and HGF gene expression. The effect of LEN on the immunomodulatory properties of hMSC was likely to be not mediated by Ikaros or Aiolos because both transcription factors were not expressed by hMSCs. These evidences underline a possible new effect of IMiDs on the alloreactivity against MM cells.

Background: Serum protein electrophoresis (SPE) and immunofixation electrophoresis (IFE) are used for diagnosis and follow-up of patients with intact immunoglobulin multiple myeloma. However, the numerous limitations of these methods led to the development of a nephelometric immunoassay (Hevylite™) for the specific measurement of serum IgGκ, IgGλ, IgAκ and IgAλ concentrations. Methods In this study, we evaluated the correlation between this assay and SPE and IFE in 114 sera of 15 patients (12 IgG and 3 IgA patients) and its impact on the clinical care of patients, especially for diagnosis, for the evaluation of residual disease and for early detection of relapse. Results At inclusion and during follow-up, we found a good correlation between monoclonal immunoglobulin concentrations and SPE (R2 = 0.902 for IgA and R2 = 0.915 for IgG) and nephelometric quantification (R2 = 0.948 for IgA and R2 = 0.920 for IgG) for the evaluation of monoclonal and polyclonal immunoglobulins. Our results illustrate that the Hevylite™ test is less sensitive than the IFE for detection of residual disease: 5 patients who obtained very good partial response or complete response had normalization of the Hevylite™ ratio while IFE was still positive. A relapse had been detectable with the Hevylite™ ratio 1 to 2 months earlier than with SPE and IFE in 3 patients out of 15, but no recommendations for treating patients with only slight biological relapse are available. Conclusion Our results demonstrate that heavy/light chain specific immunoglobulin ratios provides no additional information than serum proteins electrophoresis and immunofixation for the diagnosis and the follow-up of intact immunoglobulin multiple myeloma patients. We also studied the correlation between the concentration of total immunoglobulin measured by Hevylite™ (sum of Ig’κ + Ig’λ) and nephelometric measurement of total IgG or IgA. For this correlation analysis, all 114 sera were analyzed. The correlation coefficient was R2 = 0.948 for IgA and R2 = 0.920 for IgG

Serum free light chain (FLC) assay is an increasingly important method in diagnostic and follow-up approaches of monoclonal gammopathies. Since 2011, two immunonephelometric assays are available: the FreeliteTM assay (The Binding Site, UK) using polyclonal antibodies, and the N Latex FLC assay (Siemens, Germany) using monoclonal antibodies. While Hutchison highlighted extended reference range for the/ratio obtained with the FreeliteTM assay for patients with renal impairment (CKD-EPI<60ml/min), no modification is observed for the N Latex FLC assay as reported by Jacobs in 2014. Recent publications reported lack of correlation and few discrepancies between the 2 assays. The aim of this study was to assess clinical agreement and biological correlation between the 2 assays taking account of the renal function (CKD-EPI).

Dans le myélome multiple, les radiographies du squelette sont toujours considérées comme l’examen d’imagerie de référence car elles permettent d’établir le stade de la maladie selon la classification de Salmon et Durie. L’IRM corps entier utilisant les séquences T1 et STIR augmente la détection des lésions myélomateuses. La diffusion mesurée par IRM a démontré sa grande sensibilité en termes de détection en oncologie. L’objectif principal de cette étude était donc de comparer le bilan radiographique standard et une méthode IRM de diffusion corps entier (DWIBS) dans la détection des lésions osseuses de pathologies plasmocytaires monoclonales (myélomes multiples, leucémie à plasmocytes, plasmocytome, gammapathie monoclonale de signification indeterminée [MGUS]).Vingt-sept patients dont 24 myélomes multiples, une leucémie à plasmocytes, une MGUS et un plasmocytome ont bénéficié d’une IRM corps entier séquence DWIBS. L’IRM de diffusion et les radiographies standards, ainsi que le stade de Salmon et Durie établi par les deux méthodes ont été comparées. En cas de lésions douteuses, le suivi évolutif sur 12 mois a été utilisé comme méthode de référence au diagnostic définitif.Le taux de concordance global entre les deux techniques était de 63 %. La séquence DWIBS détectait un nombre supérieur de lésions conduisant à un stade de Salmon et Durie supérieur chez 37 % des patients : un de stade I à II, sept de stade I à III et deux de stade II à III. Chez 18,5 % des patients, l’IRM était positive alors que les radiographies étaient normales et ces discordances étaient le plus souvent situées dans les sites mal explorés par les radiographies : rachis, bassin et gril costal. Chez un patient (4 %) la séquence DWIBS donnait un stade inférieur à celui des radios (stade II vs III). Dans ce cas, les radios étaient positives au niveau des humérus et des fémurs, contrairement à la séquence DWIBS. Notre analyse site par site confirmait la nette supériorité de la séquence DWIBS par rapport aux radiographies dans l’exploration du rachis cervical (56 vs 0 % d’examens positifs, p < 0,001), dorsal (81 vs 31 %, p < 0,0002), lombaire (70 vs 35 %, p < 0,0124), du bassin (81 vs 33 %, p < 0,0005) et du gril (74 vs 36 %, p < 0,0009).L’IRM-DWIBS entraîne une majoration du stade de Salmon et Durie. Sa place dans le bilan pré thérapeutique du myélome multiple reste encore à évaluer mais cette étude semble montrer qu’il s’agit d’une méthode potentiellement intéressante.

Introduction - General practitioners report difficulties to work efficiently with hospital physicians We created a phone number dedicated to general practitioners to contact directly a hospital physician in the general internal medicine department entitled quick diagnostic and therapeutic assistance The aim of this study was to assess the first year activity of this professional support and its impact on general practitionersResults - We received 663 phone calls from February 2005 to February 2006 This led to a simple medical advice (41%) and immediate (26%) or delayed (32%) consultation or admission Results of the mail survey showed that this quick diagnostic and therapeutic assistance was helpful for the general practionersConclusion - Quick diagnostic and therapeutic assistance improve the quality of clinical care through better continuity of care between the public hospital and the general practitioners (C) 2010 Societe nationale francaise de medecine interne (SNFMI) Published by Elsevier Masson SAS All rights reserved

Le diagnostic de paralysie faciale a frigore, fréquemment porté en cas de paralysie faciale périphérique d'installation brutale, doit être néanmoins évoqué avec prudence en particulier en cas de douleur intense associée, et doit faire éliminer un processus expansif de la base du crâne, notamment du rocher.Nous rapportons le cas d'un patient de 43 ans qui a présenté une paralysie faciale périphérique de début rapidement progressif. Les examens radiologiques ont permis de mettre en évidence une tumeur du rocher, dont l'aspect radiologique pouvait faire évoquer plusieurs diagnostics, tels qu'une tumeur glomique ou une lésion secondaire. L'examen histologique a finalement établi le diagnostic de plasmocytome.La localisation de cette lésion au rocher n'a été que rarement décrite et les caractéristiques radiologiques sont très peu spécifiques. Des examens simples, tels que la recherche d'un composant monoclonal, les radiographies de squelette et le myélogramme, peuvent orienter vers le diagnostic soit de plasmocytome solitaire (lésion isolée, myélogramme normal), qui devra être confirmé par une biopsie, soit de myélome multiple (lésions ostéolytiques, myélogramme envahi), et dans ce dernier cas dispenser d'une analyse histologique.The classical hypothesis of Bell's palsy, tempting in cases of peripheral facial palsy of rapid onset, must nevertheless be evoked with caution particularly if an intense pain is present, which should lead to search for a tumor of the skull base, especially the petrous bone.A 43-year-old man presented a peripheral facial palsy of rapidly progressive onset. A petrous bone tumor was diagnosed on the CT scan, which revealed an aspect of a glomic tumor or a metastatic lesion. The final histological diagnosis was plasmacytoma.This type of tumor has been rarely reported in this location. The radiological features are not specific at all, underlying the importance of searching for some associated signs such as a monoclonal protein and performing a histological examination when the firm diagnosis of a systemic disease like multiple myeloma has not been possible.

Les catastrophes, qu'elles soient naturelles, accidentelles, ou intentionnelles touchent les bébés, les enfants et les adolescents. La reconnaissance des traumatismes psychiques est établie depuis bien longtemps chez les adultes du fait des travaux anciens chez les militaires et renouvelés dans d'autres contextes traumatiques. On reconnaît aujourd'hui l'impact de l'effraction psychique des expériences traumatiques à tous les âges de la vie et en particulier chez les très jeunes enfants qui sont le plus vulnérables du fait de leur dépendance aux adultes qui prennent soins d'eux. S'appuyant sur notre expérience au sein de la CUMP 93, nous aborderons les aspects cliniques observés chez les enfants en situation de catastrophe et les modalités d'évaluation et de prise en charge immédiates.Disasters, whether natural, accidental, or intentional, affect babies, children, and adolescents. Recognition of mental trauma has long been established in adults as a result of the work done with soldiers and renewed in other traumatic contexts. To date, we are aware of the impact of the psychic injury of traumatic experiences at all ages of life and in particular for very young children who are the most vulnerable because of their dependence on the adults who take care of them. Based on our experience in the CUMP 93, we will discuss in this communication the clinical aspects observed in children in disaster situations and the modalities for assessment and management of immediate interventions.

L’augmentation des admissions aux Urgences dépassant quotidiennement les capacités de soins a conduit les services à s’organiser afin de déterminer quels patients devaient être pris en charge rapidement et lesquels pouvaient attendre. Ce processus, dénommé « triage », consiste à déterminer à la phase initiale de la prise en charge d’un patient, la filière adaptée à son état en termes de délai et de type de soins. Cette mission est confiée en France, le plus souvent, aux infirmières d’accueil et d’orientation avec, si possible, un appel à un médecin référant dans les situations complexes et/ou les périodes de débordement. Alors qu’un tri en « très-urgent, urgent ou non urgent » s’est avéré décevant, la fiabilité de plusieurs échelles de triage en cinq priorités de prise en charge médicale a été confirmée, par une concordance entre infirmières et médecins, par une corrélation avec le recours à l’hospitalisation et par une bonne reproductibilité. Ces échelles s’appuient sur une classification des motifs de recours et, éventuellement, l’estimation des paramètres vitaux et de la douleur. L’utilité réelle du triage dans un service d’Urgences dépend des capacités des équipes à s’approprier l’échelle, à intégrer la priorité dans l’organisation des filières de soins et à mettre en place des procédures d’évaluation.As a result of increasing numbers of patients presenting to emergency departments (EDs), EDs have attempted to identify patients who need to be seen on a priority basis and patients who can wait safely. The aim of this process, named “triage”, is to assign at each patient the best channel in terms of timing and place. In France, triage is performed most-of-time by frontline nurses who can be helped by a physician for the more difficult situations or when ED is overcrowded. A simple classification “emergent, urgent, non urgent ” is unreliable but several 5-level triage scales are validated with high agreement between physicians and nurses, ability to detect admission and inter-rater & intra-rater agreements. These scales used classification of the presenting complaints, vital signs and pain. Triage usefulness depends on the team’s abilities to adopt scale, to adapt ED organization and to evaluate practice.

Les neuropathies amyloïdes liées à l'amylose AL (N-AL) et au syndrome de POEMS (N-POEMS) sont rares, associées à une gammapathie monoclonale (GM) IgGλ ou IgAλ à faible taux et des manifestations systémiques. Elles sont très invalidantes et engagent le pronostic vital.Les N-AL se présentent comme des polyneuropathies axonales longueur-dépendantes à petites fibres ou des neuropathies multifocales douloureuses et invalidantes, les N-POEMS comme une polyradiculonévrite ascendante rapide avec GM. Le diagnostic repose pour les N-AL sur la découverte de dépôts amyloïdes après biopsie nerveuse ou des glandes salivaires accessoires, de chaînes légères libres (CLL) monoclonales ; pour les N-POEMS, sur la présence de quatre critères proposés par Dispenzieri. Ces neuropathies sont associées à des biomarqueurs, utiles pour le diagnostic et le suivi de l'efficacité du traitement : taux élevé de CLL monoclonales sériques (N-AL) ou de VEGF (N-POEMS).Le diagnostic précoce de ces neuropathies et le traitement rapide par melphalan à hautes doses avec autogreffe de cellules souches hématopoïétiques (CSH) ou à faibles doses mensuelles peuvent espérer améliorer la neuropathie et leur pronostic vital.La recherche systématique de GM par immunofixation et de CLL est très utile pour la prise en charge des neuropathies périphériques évolutives d'apparence idiopathique.Primary AL amyloid polyneuropathy (AL-PN) and neuropathy due to POEMS syndrome (POEMS-N) are rare, associated with a monoclonal gammopathy (MG) IgGλ or IgAλ at a low rate and systemic manifestations. They are invalidating and life-threatening.AL-PN usually mimics small fiber length-dependent axonal polyneuropathies, but also multifocal or painful neuropathies, POEMS-N corresponds to a rapid ascending CIDP with MG. To confirm the diagnosis of AL-PN, initial investigations should identify amyloidosis on nerve or accessory salivary glands, to establish the type of amyloid after serum free light-chain (FLC) measurements. For the diagnosis of N-POEMS, diagnosis is based on the presence of four criteria proposed by Dispenzieri. These neuropathies are associated with biomarkers, useful for diagnosis and treatment monitoring: elevated serum level of FLC monoclonal in (AL-PN) or VEGF (N-POEMS).Early diagnosis of these neuropathies and early treatment using high-dose melphalan associated with an autologous hematopoietic stem cell graft or low monthly doses can improve the clinical manifestations and patient survival.Systematic search for monoclonal gammopathy by immunofixation and serum free light chains is very useful for the management of progressive peripheral neuropathies of unknown origin.