French early nationwide idecabtagene vicleucel chimeric antigen receptor T-cell therapy experience in patients with relapsed/refractory multiple myeloma (FENIX): A real-world IFM study from the DESCAR-T registry.

Idecabtagene vicleucel (ide-cel), a chimeric antigen receptor T-cell therapy targeting B-cell maturation antigen (BCMA), received early access program (EAP) authorization in France in April 2021 for relapsed/refractory multiple myeloma (RRMM). We conducted a real-world registry-based multicentre observational study in 11 French hospitals to evaluate ide-cel outcomes. Data from 176 RRMM patients who underwent apheresis between June 2021 and November 2022 were collected from the French national DESCAR-T registry. Of these, 159 patients (90%) received ide-cel. Cytokine release syndrome occurred in 90% with 2% grade ≥3, and neurotoxicity occurred in 12% with 3% grade ≥3. Over the first 6 months, the best overall response and ≥complete response rates were 88% and 47% respectively. The median progression-free survival (PFS) from the ide-cel infusion was 12.5 months, the median overall survival (OS) was 20.8 months and the estimated OS rate at 12 months was 73.3%. Patients with extra-medullary disease (EMD) had impaired PFS (6.2 months vs. 14.8 months). On multivariable analysis, EMD and previous exposure to BCMA-targeted immunoconjugate or T-cell-redirecting GPRC5D bispecific antibody were associated with inferior PFS. Our study supports ide-cel's feasibility, safety and efficacy in real-life settings, emphasizing the importance of screening for EMD and considering prior treatments to optimize patient selection.

Cessation of oral anticoagulants in antiphospholipid syndrome.

Objective To study the outcome of patients with antiphospholipid syndrome (APS) after oral anticoagulant treatment cessation. Methods We performed a retrospective study of patients with APS experiencing cessation of oral anticoagulant and enrolled in a French multicentre observational cohort between January 2014 and January 2016. The main outcome was the occurrence of recurrent thrombotic event after oral anticoagulation cessation. Results Forty four APS patients interrupted oral anticoagulation. The median age was 43 (27-56) years. The median duration of anticoagulation was 21 (9-118) months. Main causes of oral anticoagulant treatment cessation were switch from vitamin K antagonists to aspirin in 15 patients, prolonged disappearance of antiphospholipid antibodies in ten, bleeding complications in nine and a poor therapeutic adherence in six. Eleven (25%) patients developed a recurrent thrombotic event after oral anticoagulation cessation, including three catastrophic APS and one death due to lower limb ischemia. Antihypertensive treatment required at time of oral anticoagulants cessation seems to be an important factor associated with recurrent thrombosis after oral anticoagulant cessation (15.2% in patients with no relapse versus 45.5% in patients with recurrent thrombosis, p = 0.038). Oral anticoagulant treatment was re-started in 18 (40.9%) patients. Conclusion The risk of a new thrombotic event in APS patients who stopped their anticoagulation is high, even in those who showed a long lasting disappearance of antiphospholipid antibodies. Except for the presence of treated hypertension, this study did not find a particular clinical or biological phenotype for APS patients who relapsed after anticoagulation cessation. Any stopping of anticoagulant in such patients should be done with caution.

Safe and prolonged survival with long-term exposure to pomalidomide in relapsed/refractory myeloma.

BACKGROUND: The IFM2009-02 trial studied pomalidomide (4 mg daily, 21/28 versus 28/28) and dexamethasone in very advanced relapsed or refractory multiple myeloma (RRMM). We observed that 40% of patients had a prolonged progression-free survival (PFS) and subsequently overall survival (OS). We sought to analyze the characteristics of these patients and study the effect of long exposure to pomalidomide. DESIGN: We separated the studied population into two groups: 3 months to 1 year (<1 year) and more than 1 year (≥1 year) of treatment with pomalidomide and dexamethasone based on clinical judgment and historical control studies. We then analyzed the characteristics of patients according to duration of treatment. RESULTS: The overall response rate (ORR) for the <1-year group was 43%, the median PFS 4.6 months [95% confidence interval (95% CI) 3.8-6.4] with only 6% at 12 months, and the median OS was 15 months (11.7-20.3) and 40% at 18 months. For the ≥1-year group, the response rate and survival were strikingly different, ORR at 83%, median PFS 20.7 months (14.7-35.4), median OS not reached, and 91% at 18 months. CONCLUSION: Pomalidomide and dexamethasone favored prolonged and safe exposure to treatment in 40% of heavily treated and end-stage RRMM, a paradigm shift in the natural history of RRMM characterized with a succession of shorter disease-free intervals and ultimately shorter survival. Although an optimization of pomalidomide-dexamethasone regimen is warranted in advanced RRMM, we claim that pomalidomide has proven once more to change the natural history of myeloma in this series, which should be confirmed in a larger study.

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.

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 (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™ 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 R(2) = 0.948 for IgA and R(2) = 0.920 for IgG.

Peg-IFNα/ribavirin/protease inhibitor combination in hepatitis C virus associated mixed cryoglobulinemia vasculitis: results at week 24.

BACKGROUND: The standard-of-care treatment of patients with hepatitis C virus (HCV)-mixed cryoglobulinemia (MC) vasculitis includes pegylated interferon α (PegIFN)-α plus ribavirin and/or rituximab. About 30-40% of patients are non-responders or relapsers to such combination. OBJECTIVE: To analyse the safety and efficacy of Peg-IFNα/ribavirin/protease inhibitor combination in HCV-MC vasculitis. PATIENTS AND METHODS: Open-label, prospective, cohort study including 23 patients with HCV-MC vasculitis. Peg-IFNα/ribavirin was associated to telaprevir (375 mg three times daily, for 12 weeks, (n=15)) or boceprevir (800 mg three times daily, for 44 weeks, (n=8)) for 48 weeks. RESULTS: The median age was 59 (52.5-66) years, with 48.8% women. Thirteen patients (56.5%) were complete clinical responders, and 10 (43.5%) were partial responders at week 24. The virological response (ie, HCV RNA negativation) was of 69.6% at week 24 (p=0.005). The cryoglobulin level decreased from 0.44 to 0.06 g/l (p=0.0006) and the C4 level increased from 0.09 to 0.15 g/l (p=0.045). Grades 3 and 4 adverse events (mainly anaemia, neutropenia and thrombocytopenia) were observed in 10 cases (43.5%). Twenty patients (87%) received erythropoietin, 9 (39.1%) had red cell transfusion, and 2 (8.7%) had granulocyte stimulating agents. Antiviral therapy discontinuation was required in 8 (34.7%) patients for virological non-response (n=5), virological relapse (n=2) and depression (n=1). CONCLUSIONS: Peg-IFNα/ribavirin/protease inhibitor combination seems highly effective in HCV-MC. Such therapeutic regimen should be administered cautiously considering the high rate of side effects.

[Gaucher's disease in Rennes University hospital: a 10-year retrospective study]. / Etude rétrospective sur dix ans des patients atteints de maladie de Gaucher au CHU de Rennes.

INTRODUCTION: Gaucher's disease (GD) remains rare and cohort studies are essential to improve our knowledge of this disease. METHODS: We performed a 10-year retrospective study of patients with GD followed-up in the Rennes University teaching hospital. RESULTS: Among a population of 1,500,000 inhabitants, 12 patients with GD were identified. Eight were men, and four were women. Mean age at diagnosis was 32.3 years and the first symptoms appeared around 31 years old. Main symptoms were: splenomegaly (82%), hepatomegaly (64%), thrombocytopenia (73%), anemia (64%), deterioration of general status (45%), bone pain (27%). Parkinsonism was noted in two patients, polyclonal gammopathy in two others, and monoclonal gammopathy was evidenced in four patients, with chronic lymphocytic lymphoma in one of them. Enzymatic activity dosage confirmed the diagnosis of GD for eight patients. For the remaining four patients, diagnosis was obtained by identification of Gaucher's cells on tissue examination. Substitutive enzymotherapy (SE) was performed for seven patients, with great improvement of initial symptoms. For two of these seven patients, SE is changed for miglustat with persistent improvement of clinical status. CONCLUSION: Association between GD and Parkinsonism or between GD and gammopathy was confirmed in our study. Other cohort studies are needed to improve the knowledge of GD.

Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome.

Introduction: Schnitzler syndrome is an auto-inflammatory disease defined by chronic urticarial eruption and monoclonal gammopathy. 18F fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) is often performed, but its utility in Schnitzler syndrome has not been specifically investigated. The aim of this study was to determine whether PET/CT is informative in the diagnosis and follow-up of Schnitzler syndrome relative to other imaging techniques, including bone scans.Patients and methods: Patients of this study were selected from the French cohort established by Néel et al. All patients with a diagnosis of Schnitzler syndrome (according to Strasbourg's and Lipsker's criteria) who had at least one PET/CT were included. Data were collected from medical records. PET/CT scans were all reviewed by a nuclear physician blinded to the clinical and imaging data.Results: Ten patients underwent at least one PET/CT scan and all had at least one 99mTechnetium bone scan during their follow-up. The most frequent PET/CT abnormalities were diffuse bone-marrow and/or increased femoral fluorodeoxyglucose uptake, but they did not correlate with disease activity. Conversely, bone-scan abnormalities, including mainly increased radiotracer uptake in long bones, appeared to strongly correlate with Schnitzler syndrome activity.Discussion: PET/CT does not appear to be useful for the diagnosis and follow-up of Schnitzler syndrome. However, bone scans appear to be more sensitive for diagnosis and may correlate with clinical activity. Bone scans may be well positioned to distinguish Schnitzler syndrome relapse from other aetiologies of bone, joint, or muscle pain.Conclusion: Bone scans may be favoured over PET/CT in Schnitzler syndrome.

Effect of treatment on biclonal gammopathy associated with Gaucher disease.

The non-random association of Gaucher disease with polyclonal and monoclonal gammopathy has been known since 1950. The effect of treatment on monoclonal gammopathy is not well documented. We report on the long-term evolution of a biclonal gammopathy in a patient with type I Gaucher disease who was treated with splenectomy and enzyme replacement therapy. A 44-year-old man presented with hepatomegaly and massive splenomegaly. Bone marrow aspirate contained typical Gaucher cells and beta-glucosidase was low in peripheral blood leukocytes. Mutations N370S and R120W were detected. Serum protein electrophoresis disclosed two spikes in gammaglobulins. Immunofixation identified two monoclonal components: IgG kappa and IgA kappa. Gammaglobulin concentration was 31.6 g/L. A splenectomy was performed on September 2003 because of massive splenomegaly (9500 g). Two months after the splenectomy, gammaglobulin concentration was 25.2 g/L. Enzyme replacement therapy (Cerezyme 45 UI/kg every two weeks) was prescribed from April 2004 because of significant hepatomegaly and cholestasis. In April 2007 (3 years after the beginning of treatment), serum electrophoresis showed the persistence of two spikes with gammaglobulin concentration at 20.5 g/L. Simultaneously, chitotriosidase activity decreased from 6181 to 2877 nkat/L. Our observation and previous reports suggest that enzyme replacement therapy is more effective in polyclonal hypergammaglobulinaemia than in monoclonal gammopathy.

[Genetic abnormalities in multiple myeloma: role in oncogenesis and impact on survival]. / Anomalies génétiques dans le myélome: rôle dans l'oncogenèse et implications pronostiques.

PURPOSE: Recent development of interphase fluorescence in situ hybridization (FISH) allows analysis on non-proliferant plasma cells. We describe the most frequent genetic abnormalities in multiple myeloma and their prognostic value. CURRENT KNOWLEDGE AND KEY POINTS: Most frequent genetic abnormalities are illegitimate rearrangements involving the IGH gene at 14q32 (60% of patients), hyperdiploidy (50 to 60% of patients), chromosome 13 deletion (40 to -50% of patients), chromosome 1q gain (30 to -40% of patients) chromosome 17 deletion (10% of patients). Some of these genetics abnormalities are observed in monoclonal gammopathy of undetermined significance (MGUS), a pre-malignant state. t(4;14) and t(14;16) translocations and chromosome 17 deletion negatively impact the overall survival. Patients with these genomic aberrations should be treated with specific treatment. FUTURE PROSPECTS AND PROJECTS: Identification of genetic abnormalities is important for evaluation of prognosis and treatment protocol in multiple myeloma.

[Epidemiology of monoclonal gammopathy in a general hospital and a university internal medicine department]. / Epidémiologie descriptive des gammapathies monoclonales. Expérience d'un centre hospitalier général et d'un service de médecine interne de centre hospitalier et universitaire.

PURPOSE: Monoclonal gammopathy are common in the general population. We describe biological features and etiology of monoclonal gammopathy diagnosed during more than a ten year period in the Internal Medicine Department of Rennes University Hospital and in all the medical departments of General Hospital of Blois. METHODS: Patients were identified by immunofixation registry of Biochemistry Laboratories in both hospital (from 1990 in Rennes and from 1980 in Blois). RESULTS: Internal Medicine Department of Rennes University Hospital: 1051 monoclonal gammapathies were identified: 514 men and 537 women. Median age was 71. Isotypes repartition was: IgG 42.8% (450 cases), IgM 31.9% (335), IgA 8.9% (94) biclonal gammopathy 9.8% (103). Sixty-nine monoclonal light chains (6.6%) were identified. Median concentration of monoclonal protein was 14 g/l (1.8-104.4). All department of General Hospital of Blois: 1282 monoclonal gammapathies were identified: 700 men and 582 women. Median age was 79. Isotypes repartition was: IgG 59.7% (765 cases), IgM 27.5% (329), IgA 11.8% (151). Thirty-four monoclonal light chains (2.7%) were identified. Median concentration of monoclonal protein was 5.6 g/l (0.5-96.6). Most frequent diagnosis were: monoclonal gammopathy of undetermined significance or MGUS (77.6% in Blois and 64.1% in Rennes), multiple myeloma (11.9% and 12.7%), Waldenström's macroglobulinemia (4.4% and 8.7%). CONCLUSION: Monoclonal gammopathy are common in clinical practice. MGUS account for more than 60% of monoclonal gammopathy. Given their frequency, diagnostic and follow-up strategies must be costless and simple.

[Usefulness of a free light chain immunoassay in serum for the diagnosis and the follow-up of monoclonal gammopathy]. / Intérêt du dosage des chaînes légères libres d'immunoglobulines sériques pour le diagnostic et le suivi des gammapathies monoclonales.

PURPOSE: Monoclonal immunoglobulin free light chains (FLC) are present in the serum and urine of many patients with monoclonal gammopathies. In this review, we discuss the usefulness of serum FLC determination for diagnostic, prognostic and monitoring of multiple myeloma (MM), AL amyloidosis and monoclonal gammopathies of undetermined significance (MGUS). CURRENT KNOWLEDGE AND KEY POINTS: Serum FLC assay is a useful laboratory test for management of light chain MM, non-secretory MM and AL amyloidosis. Currently, serum FLC testing cannot be recommended for monitoring intact immunoglobulin multiple myeloma. Even though serum FLC determination give a better risk stratification for MGUS, systematic serum FLC assay should not be used in routine because of high MGUS occurrence in the general population. FUTURE PROSPECTS AND PROJECTS: Further prospective studies with large cohorts of patients should provide additional evidence for the role of serum FLC measurement in patients with intact immunoglobulin multiple myeloma.

[Thoracic infectious aortitis: microbiology, pathophysiology and treatment]. / Aortites thoraciques infectieuses: implications microbiologiques, physiopathologiques et thérapeutiques.

BACKGROUND: Thoracic infectious aortitis are currently rare. They are always lethal without any treatment. The microorganisms involved are numerous with particular pathophysiological characteristics for each bacterium. Treatment is difficult and must associate medical and surgical care. RECENT FINDINGS: Bacterial epidemiology of infectious aortitis has been profoundly modified with the large use of antibiotics. Syphilitic aortitis were frequent in the beginning of the twentieth century but its incidence has dramatically fallen. It still exists and its clinical presentation must be known to begin an adequate treatment. Other bacterial aetiologies of these aortitis are more classical with high frequencies of Staphylococcus aureus and Streptococcus, which are often associated with infective endocarditis. Among Gram-negative bacteria, Salmonella spp are the most frequently met microorganisms. Atherosclerosis represents the principal risk factor of these infectious aortitis. It provokes arterial parietal damage useful for bacterial attach. A saccular aneurysm of infective origin can then appear. Treatment must consist on antibiotics before surgery; Tuberculous aortitis are also possible but are much more rare. CONCLUSION: Thoracic infectious aortitis are very rare but must be known because of their poor prognosis. Treatment is difficult and prevention of atherosclerosis which is the most important risk factor of these diseases is therefore of greatest importance.

[Diagnosis of solid malignancies in an internal medicine unit: a series of 114 consecutive cases]. / Le diagnostic des cancers solides dans un service de médecine interne: étude descriptive de 114 cas consécutifs.

PURPOSE: A deterioration of the general condition, a prolonged fever or an unexplained inflammatory syndrome are frequent reasons for hospitalization in a internal medicine unit. In these situations, it is not rare to make a diagnosis of cancer. PATIENTS AND METHODS: A descriptive study was carried out over a three years period (1st October 1999 to 30th September 2002) in an internal medicine unit. Every week, all patients in whom a cancer was diagnosed were enrolled in the study. RESULTS: During this period, 165 patients were identified (3.8% of the in-patients). A histological proof was obtained in 114 patients. Digestive and bronchopulmonary cancers were the most frequent. The first signs were very varied but digestive disorders and ferriprive anaemia were the most frequent. The number of investigations necessary to diagnosis were weak (1.56 procedures) when a sign was identified but were high (5.12 procedures) when no information was provided by interview, clinical examination or usual biological tests. CONCLUSION: Diagnosis of cancer is an usual situation in an internal medicine unit. Interview and clinical examination are essential in the diagnostic step. It could decrease the number of procedures. Internal unit services are fully concerned by the announcement of cancer.

[Efficiency of rituximab in acquired hemophilia: report of two cases and review of literature]. / Efficacité du rituximab dans les hémophilies acquises: à propos de deux cas et revue de la littérature.

INTRODUCTION: Acquired hemophilia due to an inhibitor of factor VIII is a rare clinical situation. EXEGESIS: Rituximab is now used in the treatment of acquired hemophilia. We report two cases of acquired hemophilia treated by rituximab with efficiency. CONCLUSION: Rituximab appears to be a first line immunosuppressive therapy in acquired hemophilia, especially in post-partum hemophilia.

Benefit of continuous treatment for responders with newly diagnosed multiple myeloma in the randomized FIRST trial.

The phase 3, randomized Frontline Investigation of Revlimid and Dexamethasone Versus Standard Thalidomide (FIRST) trial investigating lenalidomide plus low-dose dexamethasone until disease progression (Rd continuous) vs melphalan, prednisone and thalidomide for 12 cycles (MPT) and Rd for 18 cycles (Rd18) in transplant-ineligible patients with newly diagnosed multiple myeloma (NDMM) showed that Rd continuous prolonged progression-free survival and overall survival compared with MPT. A subanalysis of the FIRST trial was conducted to determine the benefits of Rd continuous in patients with NDMM based on depth of response. Patients randomized 1:1:1 to Rd continuous, Rd18 or MPT were divided into subgroups based on best response: complete response (CR; n=290), ⩾very good partial response (VGPR; n=679), ⩾partial response (PR; n=1 225) or ⩽stable disease (n=299). Over 13% of patients receiving Rd continuous who achieved ⩾VGPR as best response did so beyond 18 months of treatment. Rd continuous reduced the risk of progression or death by 67%, 51% and 35% vs MPT in patients with CR, ⩾VGPR and ⩾PR, respectively. Similarly, Rd continuous reduced the risk of progression or death by 61%, 54% and 38% vs Rd18 in patients with CR, ⩾VGPR and ⩾PR, respectively. In patients with CR, ⩾VGPR or ⩾PR, 4-year survival rates in the Rd continuous arm (81.1%, 73.1% or 64.6%, respectively) were higher vs MPT (70.8%, 59.8% or 57.2%, respectively) and similar vs Rd18 (76.5%, 67.7% and 62.5%, respectively). Rd continuous improved efficacy outcomes in all responding patients, including those with CR, compared with fixed duration treatment.

[Syphilitic aortitis. Experience of an internal medicine unit]. / Les aortites syphilitiques. Expérience d'un service de médecine interne.

INTRODUCTION: Infectious aortitis remains a rare disease. It is characterized by an endarteritis of infectious origin generally followed by the development of a so called mycotic aneurysm. Those infectious aneurysms account for 0.5 to 1.3% of all aortic aneurysms. Of the infectious agents, Treponema pallidum has a particular place. Cardiovascular syphilitic infection was very common at the beginning of the XX(th) century with a prevalence of 6.9% of all autopsies. In 1950-1960, the prevalence had decreased to less than 1%. Since 1990, syphilis was considered as disappeared. EXEGESIS: we report syphilitic aortitis in four patients. Diagnosis, treatment, and prognosis are detailed. CONCLUSION: A syphilitic infection of the aorta should be looked for in every patient suffering from an inflammatory or infectious disease of aorta.

[Cryptococcal infections in non-HIV infected patients. Study of four cases and review of literature]. / Infections à cryptocoque chez des patients non VIH. A propos de quatre cas et revue de la littérature.

BACKGROUND: Cryptococcal infections are frequent in HIV-infected patients and are regularly looked after. This infection may occur in others immunosuppressives situations and, in those cases, diagnosis is often delayed. METHODS: We report four cases of cryptococcal infections in patients whose immunosuppression isn't related with HIV infection but due to chronic lymphocytic leukemia, giant cell temporal arteritis, gastric neoplasm and lupus. Diagnosis, prognostic and treatment are detailed. RESULTS: Four patients aged from 25 to 76 presented a cryptococcal infection (three meningitis). A woman died at the admission. Another died seven years later. The two others are still alive under treatment. When infected, all patients were immunodeficiency. CONCLUSION: Cryptococcal infection may occur in patients non-HIV-infected patients. Early detection is needed to improve prognostic.

[Diffusion weighted MRI of spine tumors]. / Evaluation de la diffusion dans la pathologie tumorale rachidienne.

PURPOSE: To evaluate the contribution of diffusion weighted MR imaging in malignant spine pathology. Materials and methods. Between February 2004 and January 2005, 49 patients (43 to 86 years old) were included. Three groups were made: osteoporotic collapses (n = 13), malignant collapses (n = 15) and malignant spine lesions (n = 21). The MRI (Symphony 1.5T) allowed SENSE imaging. After conventional MRI examination (T1, T2 fat sat, T1 with Gadolinium), all patients underwent diffusion weighted imaging (Spin Echo) with variable b values: 0, 250, 500, 750 and 1000. The diffusion sequence lasted 2 min 29 s. The Apparent Diffusion Coefficient (ADC) was calculated automatically. The analysis was qualitative (signal study b = 1,000 mm2/s) and quantitative (ADC measurement). RESULTS: The image quality was good except for some cervical examinations. Qualitative analysis did not show a difference between benign and malignant lesions. Quantitative results are: malignant spine lesion (mean ADC = 0.826 10-3 s/mm2), malignant spinal collapses (mean ADC = 0.912 10-3 s/mm2) and benign spinal collapses (mean ADC = 1.497 10-3 s/mm2). There was overlapping results between benign and malignant lesion. The statistical study showed a significant difference (t test with p < 1/10 000). For an ADC threshold value of 1.089 (malignant lesion ADC < 1.089), ROC curve showed a specificity = 80% and a sensitivity = 83.3%. CONCLUSION: Performing diffusion weighted imaging of the spine is easy with new MR technology. The ADC measurement of spine lesion provides important additional information, but does not serve as a substitute for the routine MRI sequences. In the future, it could become an important point in this difficult diagnosis.

[Pulmonary manifestations of antisynthetase syndrome]. / Les manifestations pulmonaires du syndrome des antisynthétases.

Antisynthetase syndrome is an inflammatory myopathy frequently associated with pulmonary manifestations, especially interstitial lung diseases, and uncommonly pulmonary hypertension. In the context of a suggestive clinical and radiological picture, positive anti-RNA synthetase antibodies confirm the diagnosis. Anti-Jo1, anti-PL7, and anti-PL12 antibodies are the more commonly encountered. The presence of a number of extra-thoracic manifestations in association with pulmonary disease may suggest the diagnosis. These include: myalgia or muscular deficit, Raynaud's phenomenon, polyarthritis, fever, mechanics hands. Serum creatine kinase levels are usually increased. Electromyogram, muscular magnetic resonance imaging or muscle pathology are not mandatory to make the diagnosis. There is a high variability in symptoms and severity, between patients but also during the course of the disease in the same patient. The presence of an interstitial lung disease is a major prognostic factor and an indication for more intensive treatment, principally with systemic corticosteroids with or without immunosuppressive drugs. Improving respiratory physicians' knowledge of this disease, which is often revealed by its pulmonary manifestations, should help diagnosis, therapeutic management, and possibly prognosis.

[Pomalidomide for multiple myeloma]. / Le pomalidomide dans le myélome multiple.

Once characterized by a very poor outcome, multiple myeloma (MM) now has a significantly prolonged survival, with major improvements allowed by the use of "novel agents": proteasome inhibitors (first-in-class bortezomib) and immunomodulatory compounds (IMiDs; first-in-class thalidomide and lenalidomide). However, the vast majority - if not all - of patients with MM ultimately end up being refractory to all existing drugs, including these efficient novel agents. There is a clear unmet medical need in this situation, which warrants the development of the next generation of proteasome inhibitors and IMiDs, as well as new drug classes. This review focuses on pomalidomide, the next generation IMiD, recently approved by the US FDA and the EMA for patients with relapsed or refractory MM who have received at least two prior therapies, including lenalidomide and bortezomib, and have demonstrated disease progression on their last therapy.