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Background and ObjectivesTo report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies.MethodsWe retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAPα since 2017 from 2 French referral centers.ResultsWe identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score ≤2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies.DiscussionGFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.

Objectives To describe the clinical features and outcomes of infective endocarditis (IE) in pregnant women who do not inject drugs. Methods A multinational retrospective study was performed at 14 hospitals. All definite IE episodes between January 2000 and April 2021 were included. The main outcomes were maternal mortality and pregnancy-related complications. Results Twenty-five episodes of IE were included. Median age at IE diagnosis was 33.2 years (IQR 28.3–36.6) and median gestational age was 30 weeks (IQR 16–32). Thirteen (52%) patients had no previously known heart disease. Sixteen (64%) were native IE, 7 (28%) prosthetic and 2 (8%) cardiac implantable electronic device IE. The most common aetiologies were streptococci (n = 10, 40%), staphylococci (n = 5, 20%), HACEK group (n = 3, 12%) and Enterococcus faecalis (n = 3, 12%). Twenty (80%) patients presented at least one IE complication; the most common were heart failure (n = 13, 52%) and symptomatic embolism other than stroke (n = 4, 16%). Twenty-one (84%) patients had surgery indication and surgery was performed when indicated in 19 (90%). There was one maternal death and 16 (64%) patients presented pregnancy-related complications (11 patients ≥1 complication): 3 pregnancy losses, 9 urgent Caesarean sections, 2 emergency Caesarean sections, 1 fetal death, and 11 preterm births. Two patients presented a relapse during a median follow-up of 3.1 years (IQR 0.6–7.4). Conclusions Strict medical surveillance of pregnant women with IE is required and must involve a multidisciplinary team including obstetricians and neonatologists. Furthermore, the potential risk of IE during pregnancy should never be underestimated in women with previously known underlying heart disease.

BackgroundImmune checkpoint inhibitors (ICI) have transformed cancer treatment over the last decade. Alongside this therapeutic improvement, a new variety of side effects has emerged, called immune-related adverse events (irAEs), potentially affecting any organ. Among these irAEs, myocarditis is rare but life-threatening.MethodsWe conducted a multicenter cross-sectional retrospective study with the aim of better characterizing ICI-related myocarditis. Myocarditis diagnosis was based on the recent consensus statement of the International Cardio-Oncology Society.ResultsTwenty-nine patients were identified, from six different referral centers. Most patients (55%) were treated using anti-programmed-death 1, rather than ICI combination (35%) or anti-programmed-death-ligand 1 (10%). Transthoracic echocardiography was abnormal in 52% of them, and cardiac magnetic resonance showed abnormal features in 14/24 patients (58%). Eleven patients (38%) were classified as severe. Compared with other patients, they had more frequently pre-existing systemic autoimmune disease (45% vs 6%, p=0.018), higher troponin level on admission (42-fold the upper limit vs 3.55-fold, p=0.001), and exhibited anti-acetylcholine receptor autoantibodies (p=0.001). Seven patients (24%) had myocarditis-related death, and eight more patients died from cancer progression during follow-up. Twenty-eight patients received glucocorticoids, 10 underwent plasma exchanges, 8 received intravenous immunoglobulins, and 5 other immunosuppressants. ICI rechallenge was performed in six patients, with only one myocarditis relapse.DiscussionThe management of ICI-related myocarditis may be challenging and requires a multidisciplinary approach. Prognostic features are herein described and may help to allow ICI rechallenge for some patients with smoldering presentation, after an accurate evaluation of benefit–risk balance.

Summary Background Chimeric antigen receptor (CAR) T-cell therapy can induce side-effects such as cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome (ICANS), which often require intensive care unit admission. The aim of this study was to describe management of critically ill CAR T-cell recipients in intensive care. Methods This international, multicentre, observational cohort study was done in 21 intensive care units in France, Spain, the USA, the UK, Russia, Canada, Germany, and Austria. Eligible patients were aged 18 years or older; had received CAR T-cell therapy in the past 30 days; and had been admitted to intensive care for any reason. Investigators retrospectively included patients admitted between Feb 1, 2018, and Feb 1, 2019, and prospectively included patients admitted between March 1, 2019, and Feb 1, 2020. Demographic, clinical, laboratory, treatment, and outcome data were extracted from medical records. The primary endpoint was 90-day mortality. Factors associated with mortality were identified using a Cox proportional hazard model. Findings 942 patients received CAR T-cell therapy, of whom 258 (27%) required admission to intensive care and 241 (26%) were included in the analysis. Admission to intensive care was needed within median 4·5 days (IQR 2·0–7·0) of CAR T-cell infusion. 90-day mortality was 22·4% (95% CI 17·1–27·7; 54 deaths). At initial evaluation on admission, isolated cytokine release syndrome was identified in 101 patients (42%), cytokine release syndrome and ICANS in 93 (39%), and isolated ICANS in seven (3%) patients. Grade 3–4 cytokine release syndrome within 1 day of admission to intensive care was found in 50 (25%) of 200 patients and grade 3–4 ICANS in 38 (35%) of 108 patients. Bacterial infection developed in 30 (12%) patients. Life-saving treatments were used in 75 (31%) patients within 24 h of admission to intensive care, primarily vasoactive drugs in 65 (27%) patients. Factors independently associated with 90-day mortality by multivariable analysis were frailty (hazard ratio 2·51 [95% CI 1·37–4·57]), bacterial infection (2·12 [1·11–4·08]), and lifesaving therapy within 24 h of admission (1·80 [1·05–3·10]). Interpretation Critical care management is an integral part of CAR T-cell therapy and should be standardised. Studies to improve infection prevention and treatment in these high-risk patients are warranted. Funding Groupe de Recherche Respiratoire en Reanimation Onco-Hematologique.

To assess the risk of superinfection in patients with severe COVID-19 in an ICU receiving dexamethasone with or without tocilizumab, we performed an observational cohort study. Of 246 patients, 59 developed superinfections (mainly respiratory) leading to poorer prognoses. Adding tocilizumab to dexamethasone was not associated with an increased risk of superinfection in these patients.

Background: Patients presenting with acute myeloid leukemia (AML) at diagnosis are at high risk of severe complications and death, particularly with high white blood cell (WBC) count. In this retrospective study, we evaluate interest of early and systematic support in the intensive care unit (ICU) for AML with hyperleukocytosis (AML-HL) at diagnosis. Methods: Patients with AML-HL, defined by WBC > 50 × 10 9 /L, primary referred in ICU (“Early ICU”) without organ failure and before initiating chemotherapy induction were compared to patients first admitted in the Hematology Department who required a secondary transfer in ICU ( “Late ICU” ) or not ( “No ICU” ). Primary end point was mortality during the first month, and secondary end points were the use of life-sustaining therapies in ICU and risk factors for ICU transfer and mortality. Results: One hundred fifty-four patients were included: 77 (50%) to the group “No ICU” , 18 (12%) to “Late ICU” and 59 (38%) to “Early ICU” . Mortality at day 30 was higher in “Late ICU” than in "Early ICU" and "No ICU", with 27.8%; 16.9% and 2.6% respectively (P < 0.001). "Late ICU" patients had an increased use of life-sustaining therapy comparing to "Early ICU" patients (56% vs. 29%, P = 0.04). Conclusions: Early referral to ICU reduces morbidity and seems an effective strategy to reduce short-term mortality in AML-HL at diagnosis. J Hematol. 2020;9(4):109-115 doi: https://doi.org/10.14740/jh691

Background The role of respiratory coinfections at diagnosis of Pneumocystis jirovecii pneumonia (PcP) on clinical impact has been underestimated. Methods A retrospective observational study was conducted January 2011 to April 2019 to evaluate respiratory coinfections at diagnosis of PcP patients in 2 tertiary care hospitals. Coinfection was defined by identification of pathogens from P. jirovecii-positive samples. Results Of 7882 respiratory samples tested for P. jirovecii during the 8-year study, 328 patients with diagnosis of PcP were included. Mean age was 56.7 (SD 14.9) years, 193 (58.8%) were male, 74 (22.6%) had positive HIV serology, 125 (38.1%) had viral coinfection, 76 (23.2%) bacterial coinfection, and 90-day mortality was 25.3%. In the overall population, 90-day mortality was independently associated with solid tumor underlying disease (odds ratio [OR], 11.8; 95% confidence interval [CI], 1.90–78.0; P = .008), sepsis-related organ failure assessment score (SOFA) at admission (OR, 1.62; 95% CI, 1.34–2.05; P< .001), and cytomegalovirus (CMV) respiratory coinfection (OR, 3.44; 95% CI, 1.24–2.90; P = .02). Among HIV-negative patients, respiratory CMV coinfection was associated with worse prognosis, especially when treated with adjunctive corticosteroid therapy. Conclusions Respiratory CMV coinfection at PcP diagnosis was independently associated with increased 90-day mortality, specifically in HIV-negative patients.

International audience; This study aims to assess the efficacy and safety of convalescent plasma therapy (CPT) in COVID-19 critically ill patients with protracted severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) RNAemia. A retrospective cohort study was conducted in intensive care unit (ICU). All patients with severe COVID-19 pneumonia for whom RNAemia remained positive more than 14 days after onset of the infection were included and given CPT. The primary objective was to evaluate SARS-CoV-2 RNAemia 7 days (D7) after CPT. A total of 14 patients were included and they received a median CPT volume of 828 ml (range: 817-960). CPT was administered in a median time of 14 days after ICU admission. At D7, 13/14 patients had negative SARS-CoV-2 blood PCR and one patient had negative blood PCR 11 days after CPT. At D7 and at D14, the clinical status was improved in 7/14 and 11/14 patients, respectively. The 28-day mortality rate was 14%. No CPT-related adverse effects had been reported. CPT is safe and may be efficient in patients with protracted RNAemia admitted in ICU for severe COVID-19 pneumonia. Randomized controlled trials are needed to confirm these results.

Contexte La transfusion de plasma de donneurs convalescents (PDC) au cours de la COVID-19 est controversée. En France, hors essai clinique, elle est encadrée par un protocole à usage thérapeutique soumis à un avis multidisciplinaire (RCP) préalable. L’expérience de ce traitement en réanimation n’est pas décrite. Méthode Tous les patients admis dans une unité de réanimation d’un CHU pour un SDRA COVID-19 et ayant une PCR plasmatique SARS-CoV-2 positive au moins 10 jours après le début des symptômes ont reçu une transfusion de PDC après validation en RCP. L’objectif principal était d’évaluer la tolérance et la virémie 7 j après la transfusion (J7). Résultats Entre mai 2020 et avril 2021, 31 patients (27 hommes et 4 femmes, âge moyen = 69 ans) ont été inclus et ont reçu un volume médian de 836 mL de PDC sur 2 jours consécutifs. Tous présentaient au moins un facteur de risque de forme grave dont 15 une affection maligne évolutive et 14 avaient une sérologie SARS-CoV-2 négative à l’admission en réanimation. Les PDC ont été transfusés en moyenne 18 j après le début des symptômes et 8 j après l’admission en réanimation. À J7, 22/31 patients avaient une PCR plasmatique négative. Le délai médian de négativation de la PCR plasmatique était de 8 j. À J 7, le score de sévérité clinique OMS était stable pour 12/31 (39 %), en amélioration pour 14/31 (45 %) et détérioré pour 5/31 (16 %) patients. La mortalité à 28 jours était de 26 % (8/31 dont 3 dans les sept jours après PDC). Aucun évènement indésirable significatif lié à la transfusion n’a été rapporté. Conclusion Notre étude confirme la faisabilité de la transfusion de PDC en réanimation, notamment en cas d’immunodépression humorale.

International audience; PURPOSE: High-resolution free-breathing late gadolinium enhancement (HR-LGE) was shown valuable for the diagnosis of acute coronary syndromes with non-obstructed coronary arteries. The method may be useful to detect COVID-related myocardial injuries but is hampered by prolonged acquisition times. We aimed to introduce an accelerated HR-LGE technique for the diagnosis of COVID-related myocardial injuries. METHOD: An undersampled navigator-gated HR-LGE (acquired resolution of 1.25 mm(3)) sequence combined with advanced patch-based low-rank reconstruction was developed and validated in a phantom and in 23 patients with structural heart disease (test cohort; 15 men; 55 ± 16 years). Twenty patients with laboratory-confirmed COVID-19 infection associated with troponin rise (COVID cohort; 15 men; 46 ± 24 years) prospectively underwent cardiovascular magnetic resonance (CMR) with the proposed sequence in our center. Image sharpness, quality, signal intensity differences and diagnostic value of free-breathing HR-LGE were compared against conventional breath-held low-resolution LGE (LR-LGE, voxel size 1.8x1.4x6mm). RESULTS: Structures sharpness in the phantom showed no differences with the fully sampled image up to an undersampling factor of x3.8 (P > 0.5). In patients (N = 43), this acceleration allowed for acquisition times of 7min21s ± 1min12s at 1.25 mm(3) resolution. Compared with LR-LGE, HR-LGE showed higher image quality (P = 0.03) and comparable signal intensity differences (P > 0.5). In patients with structural heart disease, all LGE-positive segments on LR-LGE were also detected on HR-LGE (80/391) with 21 additional enhanced segments visible only on HR-LGE (101/391, P

COVID‐19 leads to mortality of several patients and the cytokine storm is reportedly critical in the patients. In order to reduce the cytokine storm, we would like to propose the IL6 receptor antagonist therapy for the COVID‐19 patients. Two humanized monoclonal antibodies are in clinical trial following IL6R antagonist therapies namely tocilizumab and sarilumab. However, researchers and physicians should look for more IL6 receptor antagonists for the therapy of cytokine storm syndrome SARS‐CoV‐2 infected persons to enhance the therapeutic options for cytokine storm. This article is protected by copyright. All rights reserved.

Background Coronavirus disease 2019 (COVID-19)-associated acute kidney injury (AKI) frequency, severity and characterization in critically ill patients has not been reported. Methods Single-centre cohort performed from 3 March 2020 to 14 April 2020 in four intensive care units in Bordeaux University Hospital, France. All patients with COVID-19 and pulmonary severity criteria were included. AKI was defined using Kidney Disease: Improving Global Outcomes (KDIGO) criteria. A systematic urinary analysis was performed. The incidence, severity, clinical presentation, biological characterization (transient versus persistent AKI; proteinuria, haematuria and glycosuria) and short-term outcomes were evaluated. Results Seventy-one patients were included, with basal serum creatinine (SCr) of 69 ± 21 µmol/L. At admission, AKI was present in 8/71 (11%) patients. Median [interquartile range (IQR)] follow-up was 17 (12–23) days. AKI developed in a total of 57/71 (80%) patients, with 35% Stage 1, 35% Stage 2 and 30% Stage 3 AKI; 10/57 (18%) required renal replacement therapy (RRT). Transient AKI was present in only 4/55 (7%) patients and persistent AKI was observed in 51/55 (93%). Patients with persistent AKI developed a median (IQR) urine protein/creatinine of 82 (54–140) (mg/mmol) with an albuminuria/proteinuria ratio of 0.23 ± 20, indicating predominant tubulointerstitial injury. Only two (4%) patients had glycosuria. At Day 7 after onset of AKI, six (11%) patients remained dependent on RRT, nine (16%) had SCr >200 µmol/L and four (7%) had died. Day 7 and Day 14 renal recovery occurred in 28% and 52%, respectively. Conclusion Severe COVID-19-associated AKI is frequent, persistent, severe and characterized by an almost exclusive tubulointerstitial injury without glycosuria.

Current guidelines recommend cefazolin as an alternative to antistaphylococcal penicillins (ASPs) in methicillin-susceptible Staphylococcus aureus (MSSA) infective endocarditis despite the lack of comparative study. The objective of this study was to evaluate the comparative outcomes of cefazolin vs. ASPs in MSSA infective endocarditis.This was a retrospective analysis of an observational multicentre cohort study using prospectively collected data from patients with MSSA endocarditis confirmed by endocarditis team and treated either with cefazolin or ASPs between July 2013 and December 2018. Patients were excluded if they received both treatments. The primary outcome was 90-day all-cause mortality.Of 210 patients included, 53 patients (25.2%) received cefazolin and 157 (74.8%) received ASPs. The overall 90-day mortality rate was 27.6% (58/210 patients), 24.5% (13/53) in the cefazolin group vs. 28.7% (45/157) in the ASP group (p 0.561). Premature antimicrobial discontinuation due to adverse events occurred less frequently with cefazolin than with ASPs (0/53 vs. 13/157 patients; p 0.042). In multivariate analysis, there was no difference in 90-day mortality between cefazolin and ASPs (adjusted odds ratio (aOR), 1.2; 95% confidence interval (CI), 0.49-2.91; p 0.681), while age (aOR, 1.06; 95% CI, 1.03-1.09; p 0.001), Charlson comorbidity index (aOR, 1.18; 95% CI, 1.02-1.36 p 0.023), cerebral embolism (aOR, 2.83; 95% CI, 1.33-6.14; p 0.007) and intensive care unit admission (aOR, 4.16; 95% CI, 1.89-9.59; p 0.001) were factors significantly associated with higher mortality.Cefazolin seems to be a possible alternative to ASPs in MSSA endocarditis. More studies are needed to confirm these results and determine which treatment should be recommended as first-line therapy.

BackgroundCOVID-19-associated acute kidney injury frequency, severity and characterisation in critically ill patients has not been reported.MethodsSingle-center cohort performed from March 3, 2020, to April 14, 2020 in 4 intensive care units in Bordeaux University Hospital, France. All patients with COVID19 and pulmonary severity criteria were included. AKI was defined using KDIGO criteria. A systematic urinary analysis was performed. The incidence, severity, clinical presentation, biological characterisation (transient vs. persistent acute kidney injury; proteinuria, hematuria and glycosuria), and short-term outcomes was evaluated.Results71 patients were included, with basal serum creatinine of 69 ± 21 µmol/L. At admission, AKI was present in 8/71 (11%) patients. Median follow-up was 17 [12–23] days. AKI developed in a total of 57/71 (80%) patients with 35% Stage 1, 35% Stage 2, and 30% Stage 3 acute kidney injury; 10/57 (18%) required renal replacement therapy. Transient AKI was present in only 4/55 (7%) patients and persistent AKI was observed in 51/55 (93%). Patients with persistent AKI developed a median urine protein/creatinine of 82 [54–140] (mg/mmol) with an albuminuria/proteinuria ratio of 0.23 ± 20 indicating predominant tubulo-interstitial injury. Only 2 (4%) patients had glycosuria. At Day 7 onset of after AKI, six (11%) patients remained dependent on renal replacement therapy, nine (16%) had SCr > 200 µmol/L, and four (7%) died. Day 7 and day 14 renal recovery occurred in 28% and 52 % respectively.ConclusionCOVID-19-associated AKI is frequent, persistent severe and characterised by an almost exclusive tubulo-interstitial injury without glycosuria.

Background In the management of infective endocarditis (IE), the presence of extracardiac complications has an influence on both diagnosis and treatment. Current guidelines suggest that systematic thoracoabdominal-pelvic computed tomography (TAP-CT) may be helpful. Our objective was to describe how systematic TAP-CT affects the diagnosis and the management of IE. Methods In this multicenter cohort study, between January 2013 and July 2016 we included consecutive patients who had definite or possible IE according to the Duke modified criteria, validated by endocarditis teams. We analyzed whether the Duke classification and therapeutic management were modified regarding the presence or the absence of IE-related lesion on CT and investigated the tolerance of this examination. Results Of the 522 patients included in this study, 217 (41.6%) had 1 or more IE-related lesions. On the basis of CT results in asymptomatic patients, diagnostic classification was upgraded from possible endocarditis to definite endocarditis for only 4 cases (0.8%). The presence of IE-related lesions on CT did not modify the duration of antibiotic treatment (P = .55), nor the decision of surgical treatment (P = .39). Specific treatment of the lesion was necessary in 42 patients (8.0%), but only 9 of these lesions (1.9%) were asymptomatic and diagnosed only on the TAP-CT. Acute kidney injury (AKI) within 5 days of CT was observed in 78 patients (14.9%). Conclusions The TAP-CT findings slightly affected diagnosis and treatment of IE in a very small proportion of asymptomatic patients. Furthermore, contrast media should be used with caution because of the high risk of AKI.

Introduction La cefazoline a ete montree comme etant une alternative efficace aux penicillines anti-staphylococciques (PAS) mais uniquement dans les bacteriemies a Staphylocoque dore sensible a la meticilline (SASM). Pourtant, les recommandations actuelles americaines et europeennes recommandent la cefazoline en alternative aux PAS dans l’endocardite infectieuse alors meme que cette molecule pourrait etre concernee par un effet inoculum. L’objectif de cette etude etait donc de comparer l’efficacite entre la cefazoline et les PAS dans les endocardites a SASM. Materiels et methodes Dans cette etude de cohorte bicentrique, tous les patients dont le diagnostic d’endocardite a SASM a ete retenu en reunion de concertation pluridisciplinaire endocardite entre juillet 2013 et decembre 2018 ont ete screenes. Les patients etaient inclus s’ils avaient recu soit un traitement par cefazoline, soit un traitement par PAS. Le critere de jugement principal etait la mortalite toute cause a 90 jours (J90). Les criteres de jugements secondaires etaient les emboles symptomatiques sous traitement, la rechute, la duree de la bacteriemie et les arrets de traitement pour effets indesirables. Resultats Dans cette etude, 225 patients ont ete inclus, 182 (72,0 %) dans le groupe PAS et 63 (28 %) dans le groupe cefazoline. La duree mediane de traitement etait la meme dans les deux groupes (32 jours). La mortalite globale a J90 etait de 28,4 % (64 sur 225 patients). Il n’y avait pas de difference de mortalite entre le groupe cefazoline et le groupe PAS (p = 0,91) en analyse multivariee. En revanche, l’âge (p Conclusion Nous n’avons pas retrouve de difference d’efficacite ou de tolerance entre cefazoline et penicilline anti-staphylococcique pour le traitement des endocardites a SASM. Les facteurs associes a une sur-mortalite ainsi que les facteurs protecteurs etaient ceux classiquement retrouves dans la litterature.

In nonoperated prosthetic valve endocarditis (PVE), long-term outcome is largely unknown. We report the follow-up of 129 nonoperated patients with PVE alive at discharge. At 1 year, the mortality rate was 24%; relapses and reinfection were rare (5% each). Enterococcal PVE was associated with a higher risk of relapse.

Introduction Nous presentons le cas d’une patiente de 26 ans immunodeprimee a la suite d’une chimiotherapie, developpant une polyarthrite septique a plusieurs germes pyogenes successifs, s’aggravant sous antibiotherapies adaptees. Le dernier d’entre eux, tres inhabituel dans les articulations, n’a pu etre identifie que grâce a la PCR 16S apres 4 mois d’errance diagnostique, permettant l’introduction d’un traitement efficace. Observation Une patiente de 26 ans etait en cours de chimiotherapie par carboplatine et etoposide pour un thymome associe a une myasthenie traitee initialement par rituximab puis azathioprine. Elle developpait une mono-arthrite de cheville droite non ponctionnable dans les suites d’une infection de chambre implantable associee a une bacteriemie a Staphylococcus aureus Meti-Sensible. Malgre l’ablation de la chambre et une antibiotherapie adaptee, la fievre persistait et une arthrite de hanche se developpait. Deux lavages articulaires de la hanche droite realises a un mois d’intervalle retrouvaient un Staphylococcus capitis sur les deux prelevements et un Enterobacter cloacae sur le deuxieme. Malgre les differentes lignes d’antibiotiques (cefazoline, rifampicine, meropeneme, clindamycine, daptomycine), la patiente demeurait febrile pendant 4 mois, majorait son syndrome inflammatoire et de nouvelles localisations articulaires apparaissaient (pied droit, acromio-claviculaire gauche, genou gauche). Les nouvelles ponctions articulaires etaient steriles malgre le liquide purulent (215 000 polynucleaires neutrophiles/mm3). On notait par ailleurs une hypogammaglobulinemie a 5,6 g/L. Le bilan de fievre prolongee ne retrouvait pas de germe atypique ni de cause dysimmunitaire. Les hemocultures et l’echographie trans-œsophagienne etaient normales. Finalement, la PCR16S revenait positive sur la ponction articulaire de genou a Ureaplasma parvum, confirmee par la culture sur milieu de Shepard et la PCR specifique. Apres lavage articulaire du genou gauche et instauration de Doxycycline 200 mg/j, l’apyrexie etait obtenue en 5 jours. La patiente a ainsi ete traitee pendant 6 mois avec une excellente evolution infectieuse et articulaire mais des sequelles fonctionnelles. Discussion U. parvum est une bacterie intracellulaire stricte de croissance difficile appartenant a la famille des mycoplasmes d’origine urogenitale. Ce germe a la particularite de necessiter une culture specifique, ainsi que d’etre resistant aux antibiotherapies ciblant les bacteries extracellulaires. L’infection a mycoplasme est une cause d’arthrite septique chez les patients hypogammaglobulinemiques avec une prevalence jusqu’a 38 % des arthrites septiques dans cette population. Elle a aussi ete decrite chez des patients transplantes ou atteints de lymphome. La demande de culture specifique (Shepard) de cette bacterie semble donc pertinente devant toute polyarthrite indeterminee chez un patient hypogammaglobulinemique. Dans la litterature, l’espece la plus retrouvee d’Urea plasma au niveau articulaire est Ureaplasma urealyticum. Nous presentons ici la quatrieme arthrite publiee a U. parvum. Dans notre cas, c’est la PCR « universelle » 16S qui a permis de faire le diagnostic, ce qui confirme son interet dans les arthrites d’allure septique a culture standard negative. Conclusion Le diagnostic d’arthrite a mycoplasme doit etre evoque devant une polyarthrite d’allure septique non documentee, d’autant plus chez un patient ayant une hypogammaglobulinemie.

Glial fibrillary acidic protein is a recently identified rare cause of autoimmune encephalomyelitis, in which the cerebrospinal fluid shows lymphocytic pleocytosis accompanied by linear perivascular radial gadolinium enhancement in the brain. We report a 19-year-old man admitted to the intensive care unit with suspected viral meningoencephalitis. Magnetic resonance imaging showed mild encephalopathy with a reversible splenial lesion. He quickly developed a coma and acute respiratory failure. Glial fibrillary acidic protein antibodies and human parainfluenza virus were detected by cerebrospinal fluid exams. He was treated with intravenous immunoglobulin, methylprednisolone pulses, plasma exchange and then six infusions of cyclophosphamide plus two of rituximab, which resulted in a total recovery.

International audience

In the last decades, the number of cancer patients admitted in intensive care units (ICUs) for septic shock has dramatically increased. However, prognosis data remain scarce. To assess the 180-day mortality rate in cancer patients admitted to the ICU for septic shock, a 5-year prospective study was performed. All adult patients admitted for septic shock were included and categorized into the following two groups and four subgroups: cancer patients (solid tumor or hematological malignancy) and non-cancer patients (immunocompromised or not). Data were collected and compared between the groups. Upon early ICU admission, the decision to forgo life-sustaining therapy (DFLST) or not was made by consultation among hematologists, oncologists, and the patients or their relatives. During the study period, 496 patients were admitted for septic shock: 252 cancer patients (119 hematological malignancies and 133 solid tumors) and 244 non-cancer patients. A DFLST was made for 39% of the non-cancer patients and 52% of the cancer patients. The 180-day mortality rate among the cancer patients was 51% and 68% for those with hematological malignancies and solid cancers, respectively. The mortality rate among the non-cancer patients was 44%. In a multivariate analysis, the performance status, Charlson comorbidity index, simplified acute physiology score 2, sequential organ failure assessment score, and DFLST were independent predictors of 180-day mortality. Despite early admission to the ICU, the 180-day mortality rate due to septic shock was higher in cancer patients compared with non-cancer patients, due to excess mortality in the patients with solid tumors. The long-term prognosis of cancer patients with septic shock is modulated by their general state, severity of organ failure, and DFLST.

Resume Introduction L’hyperammoniemie attribuee au myelome est rarement decrite. Observation Nous rapportons l’observation d’un patient de 63 ans admis en reanimation medicale pour confusion et troubles de la conscience en rapport avec une hyperammoniemie attribuee a une recidive de son myelome. La chimiotherapie permettait la diminution de l’ammoniemie et le retour a un etat de conscience normal. Conclusion L’encephalopathie hyperammoniemique est une complication rare du myelome, associee a un fort taux de mortalite. A notre connaissance, il s’agit du premier cas de recidive de myelome diagnostique et traite en reanimation.

Guidelines for preventing Pneumocystis pneumonia (PCP) in HIV patients are based on CD4 below 200/mm3. Such cut-off value is suggested to guide prophylaxis in non-HIV conditions (NHIV) especially in autoimmune and inflammatory diseases (AD). We aimed to determine if CD4 could be used to guide PCP prophylaxis in AD.CD4 and lymphocyte-count were retrospectively studied in patients diagnosed with PCP between January 2013 and February 2016.129 patients were included. The median CD4-count was 302/mm3 in AD, which was significantly higher than in HIV patients (19/mm3; p0.0001). Fifty percent (n=10) of AD patients had CD4 counts greater than 300/mm3.Prophylaxis for PCP cannot rely solely on CD4-count in NHIV patients especially in AD.

We describe the effect of interleukin 6 (IL-6) blockade using tocilizumab (TCZ) for inducing and maintaining remission of refractory polyarteritis nodosa (PAN). Three patients with refractory PAN defined according to the American College of Rheumatology criteria were treated with TCZ infusions (8 mg/kg) on a monthly basis. All of them had severe cutaneous and articular involvement with elevated biological inflammatory markers. One suffered from a neuritis multiplex and one from renal and digestive damage. All three patients were dependent on high doses of glucocorticoids (above 0.5 mg/kg) and two of them were resistant to immunosuppressive drugs. All patients achieved and maintained clinical response and normalisation of the inflammation acute-phase proteins after a few weeks of treatment with TCZ. Prednisolone could be reduced by an average of 41–13 mg/day. These first case reports suggest that IL-6 blockade using TCZ could be a therapeutic alternative to induce remission in patients with polyarteritis nodosa resistant or intolerant to the reference treatment.

Resume Introduction La leptospirose est une anthropozoonose due a un spirochete, Leptospira interrogans , dont l’expression clinique est polymorphe. La thrombopenie est un signe biologique classique a la phase aigue de l’infection, dont le mecanisme de survenue est mal connu. Observation Nous rapportons l’observation d’un homme de 56 ans, hospitalise pour sepsis severe associe a une insuffisance renale aigue et une insuffisance hepatocellulaire. Compte tenu de la recente inondation de sa maison, le diagnostic de leptospirose etait rapidement pose. Un myelogramme etait realise en raison d’une thrombopenie a 9 G/L associee a une hyperferritinemie et une hypertriglyceridemie. Il mettait en evidence de nombreux megacaryocytes et des images d’hemophagocytose confirmant le diagnostic de syndrome d’activation macrophagique. Les anomalies biologiques se corrigeaient parallelement a la guerison de l’infection. Conclusion Nous suggerons que la destruction centrale des precurseurs par hemophagocytose est un mecanisme possible de la thrombopenie lors de la leptospirose dont le diagnostic repose sur la realisation d’un myelogramme.

Resume Notre but a ete de comparer les huit biotherapies disponibles pour le traitement de la polyarthrite rhumatoide dans deux populations differentes de patients, les patients naifs au methotrexate et ceux ayant eu une reponse inadequate au methotrexate, en se basant sur une revue extensive de la litterature. Les cinq anti-TNFα, le rituximab, l’abatacept et le tocilizumab utilises en association au methotrexate ont ete compares au methotrexate en monotherapie en utilisant la reponse ACR50 comme objectif primaire ainsi que l’absence de progression structurale a 1 an de la premiere evaluation radiographique. Les odds ratios (OR) ont ete calcules de meme que le nombre de sujets necessaires de traiter (Number Needed to Treat [NNT]) afin d’obtenir une reponse ACR50 pour chaque biotherapie. Nous avons inclus 22 etudes. Au total, l’association d’une biotherapie etait significativement plus efficace que le methotrexate seul, que ce soit dans le groupe des patients naifs au methotrexate (OR, 2,11 ; indice de confiance 95 % (IC95 %), 1,85–2,41) ou dans le groupe de reponse inadequate au methotrexate (OR, 4,82 ; IC95 %, 3,83–6,08). Les NNT bruts etaient les suivants : etanercept, cinq dans le groupe naif et trois dans le groupe de reponse inadequate ; adalimumab, sept et trois ; infliximab, sept et cinq ; abatacept, sept et quatre ; rituximab, cinq et cinq ; et tocilizumab et certolizumab, quatre dans le groupe reponse inadequate. Aucune difference significative n’a ete notee entre les biotherapies. Dans le groupe naif, le traitement combine etait associe a une plus grande frequence de l’absence de progression structurale apres un an compare au methotrexate seul (OR, 2,19 ; IC95 %, 1,55–3,08). Toutes les biotherapies avaient approximativement la meme efficacite. Les patients naifs de methotrexate traites par biotherapie avaient significativement moins de progression radiographique que ceux sous methotrexate.

Background The incidence of embolic events (EE) is high in patients with infective endocarditis (IE). EE influence patient management in different settings because they are minor criteria in the Duke classification and may lead to changes medical therapy or surgical strategy. If current guidelines suggest that systematic thoraco-abdominopelvic CT scan (TAP-CT) may be helpful, reliable data are lacking. The main objective of this study was to describe how systematic TAP-CT affects the diagnosis of patients with IE. Secondary objectives were to assess the impact of the TAP-CT on the management of patients with IE and the incidence of contrast-induced acute kidney injury (CI-AKI). Methods In this multicenter cohort study between January 2013 and July 2016, we included consecutive patients who had definite or possible acute IE according to the Duke-modified criteria, and after validation by the endocarditis teams. The main exclusion criterion was the absence of TAP-CT scan. We compared the Duke classification diagnosis data and treatment data (medical and/or surgical) regarding the presence or the absence of EE on the CT and investigated the tolerance of this examination as well. Results Of the 522 patients included in this study, 217 (41.6%) had one or more EE on the TAP-CT. The two major Duke modified criteria were found in 397 patients (76,1%) and 457 patients (87,6%) had a definite endocarditis. On the basis of TAP-CT results in asymptomatic patients, diagnostic classification was upgraded from possible endocarditis to definite endocarditis for only four cases which represent 0.8% of the population. The presence of EE on CT did not modify the duration of antibiotic treatment (P = 0.55) and the decision of surgical treatment (P = 0.39). Specific treatment of the EE was necessary in 42 patients (8.0%) but only nine of these EE (1.9%) were asymptomatic. CI-AKI was observed in 78 patients (14.9%). Conclusion The CT-scan findings slightly affected diagnosis of IE. The impact on the therapeutic management is low and the incidence of CI-AKI should not be underestimated. Additional studies are needed to assess whether CT-scan improves patient outcomes, leads to unnecessary procedures and increased costs. Disclosures All authors: No reported disclosures.

Background The management of infective endocarditis (IE) by an expert medico-surgical team through multidisciplinary consultation meetings is now recommended. While it seems clear that it can improve the short-term prognosis of patients, long-term data are still scarce. Methods All patients hospitalized between 2013 and 2017 in the three teaching hospitals of our center with an IE treated by the multidisciplinary team were followed prospectively at 1, 3, 6, and 12 months. The main objective was to determine the 1-year mortality of the entire cohort treated by the team. Results During the study, 493 patients had a certain or possible IE and the outcome at 1 year was known for 448 of them (4 lost to follow-up and 41 followed for less than 1 year): 254 had native valve IE (57%) and 194 had prosthetic valve IE (43%). The median age of IE patients was 69.3 years (155 patients were over 75 years old) and 329 (73%) were men. Healthcare-associated IE (HAIE) accounted for 47% of cases. A microorganism was isolated in 92% of cases (S. aureus = 24%), 252 patients (56%) had an embolic events and 68 (15%) had heart failure. The Charlson Median Comorbidity Index (ICC) was 5.0. Two hundred sixteen patients (48%) underwent surgery. The mortality rates at 1 month, 3 months, 6 months, and 1 year were, respectively, 14.1%, 19.0%, 23.2%, and 27.7%. The ICC at inclusion of patients who died at 1 year was 6.0 vs. 4.0 for survivors. Mortality at 1 year was significantly higher in case of HAIE (33% vs. 23%), documented S. aureus IE (39% vs. 24%), exclusive medical treatment (40% vs. 15%), and heart failure (43% vs. 25%). Conclusion While the management of IE by an endocarditis team seems to improve the short-term prognosis of IE, 1-year mortality remains high as patients are increasingly older and have severe comorbidities. Our study confirms that early prognostic factors remain in the long term and that the prognosis is better in community-acquired IE with surgery. Disclosures All authors: No reported disclosures.

Introduction Les dermatomyosites sont des pathologies a haut risque de pneumocystose parmi les maladies auto-immunes [1] . Nous rapportons ici le cas d’une pneumocystose developpee chez une patiente avec une authentique dermatomyosite a anticorps anti-MDA5. Observation Une femme de 57 ans est admise en reanimation pour un tableau de detresse respiratoire d’installation subaigue. Depuis 6 semaines elle est traitee par prednisone (1 mg/kg, 80 mg/jour) et azathioprine (100 mg/jour) pour un tableau de dermatomyosite avec anticorps anti-MDA5. Des le diagnostic elle presente un tableau de myalgies, atteinte cutanee (papules de Gottron), et atteinte pulmonaire interstitielle sans retentissement sur les EFR (DLCO non faite). Elle n’a pas d’autre antecedent significatif. Apres une amelioration clinique rapide, la patiente presente apres un mois de traitement une toux avec dyspnee. Les images interstitielles se majorent. Des IGIV (2 g/kg) sont ajoutees au traitement et la patiente est alors hospitalisee en medecine. Devant l’aggravation respiratoire, elle est transferee en reanimation. A son arrivee, la saturation est a 98 % sous 4 L/min, TA 130/90. Elle est subfebrile a 38°, les CPK sont a 2 N, la CRP a 122 mg/L. Les CD4 sont a 545/mm3, les lymphocytes totaux a 856/mm3. Les images interstitielles avec notamment de grandes plages de verre depoli se majorent nettement sur un nouveau scanner thoracique. La PCR Pneumocystis jiroveci sur ECBC est positive a 76 000 cp/mL avec un examen direct negatif, un LBA est realise et met en evidence une alveolite lymphocytaire (400 000 elts/mm3, 21 %) avec examen direct positif pour P. jiroveci. Un traitement par cotrimoxazole est rapidement debutee. La situation se degrade rapidement avec un tableau de SDRA puis de defaillance multiviscerale necessitant ventilation mecanique et epuration extrarenale. L’atteinte cutanee progresse avec de nouvelles lesions ainsi que l’atteinte musculaire avec une elevation des CPK. Malgre une majoration de la corticotherapie (3 bolus a 250 mg puis 2 mg/kg), la patiente decede. Discussion Les dermatomyosites a anticorps anti-MDA5 sont une entite recente dont l’atteinte pulmonaire est particulierement severe. Plusieurs cas de pneumocystose associees a une dermatomyosites a MDA5 ont deja ete rapportees et compliquees de deces [2] . La gravite de l’atteinte interstitielle, la corticotherapie generale et les perturbations du systeme immunitaire sont les facteurs de risque principaux de pneumocystose dans cette pathologie. Conclusion Une prophylaxie systematique par cotrimoxazole doit etre discutee dans le cas des dermatomyosites a anticorps anti-MDA5 et plus particulierement si une corticotherapie superieure a 20 mg/jour est instauree, meme si le taux de CD4 n’est pas abaisse.

Introduction L’epidemiologie de la pneumocystose (PCP) s’est modifiee ces dernieres annees et touche desormais preferentiellement des patients non infectes par le VIH et exposes a divers immunosuppresseurs (IS) [1] . Chez le sujet VIH, l’indication d’une prophylaxie medicamenteuse est bien codifiee [2] . Malheureusement, il n’existe pas de recommandations aussi claires dans d’autres contextes [3] . Nous avons voulu analyser le profil clinicobiologique des patients atteints de pathologies inflammatoires et auto-immunes (MAI) ayant une PCR Pneumocystis positive. Patients et methodes Notre etude retrospective porte sur des patients admis au CHU de Bordeaux entre janvier 2013 et janvier 2016 et ayant une qPCR ( quantitative polymerase chain reaction ) positive pour Pneumocystis jirovecii (PJ). Les patients ont ete classes retrospectivement en trois groupes : PCP certaine, possible et colonisation. Ces groupes ont ete constitues independamment des valeurs de PCR, a partir des donnees cliniques, du resultat de l’examen direct des secretions respiratoires, de l’imagerie et du traitement administre. Resultats Cent-cinquante patients avaient au moins une PCR PJ positive, dont 39 patients VIH, 35 avec une hemopathie maligne, 26 greffes d’organes, 10 cancers solides, 7 avec des pathologies diverses et 33 avec une MAI (groupe intitule « medecine interne »). Notre etude porte sur ce groupe « medecine interne ». Le ratio H/F etait de 1,54, l’âge moyen de 66 ans . Six colonisations, 15 PCP certaines, et 12 PCP possibles etaient identifiees. Les pathologies sous-jacentes etaient respectivement : polyarthrite rhumatoide ( n = 7 dont 2 avec vascularite), dermato/polymyosite ( n = 4), vascularites a ANCA ( n = 3), hepatite auto-immune ( n = 3), maladie de Horton/pseudopolyarthrite rhizomelique ( n = 3), cryoglobulinemie ( n = 3), pneumopathie interstitielle diffuse ( n = 2), nephropathie glomerulaire ( n = 2), anemie hemolytique auto-immune ( n = 1), hemophilie A acquise ( n = 1), spondylarthrite ankylosante( n = 1), sclerodermie ( n = 1), sarcoidose ( n = 1), myasthenie ( n = 1). Le delai moyen entre l’episode de PCP suspecte et le diagnostic de la MAI etait de 5,6 ans (0–28 ans). Une corticotherapie (CTC) etait prescrite chez 97 % des patients avant l’admission, depuis en moyenne 3,4 ans (19 jours–24 ans) a la dose quotidienne moyenne de 29,4 mg (5–80 mg). Une dose > 20 mg/jour etait retrouvee dans 64 % des cas. Neuf patients (27 %) recevaient une CTC seule sans autre IS associe. Vingt-trois patients (70 %) avaient recu en association a une CTC un traitement IS dans les 3 mois precedents (methotrexate : 21 %, azathioprine : 12 %, rituximab : 12 %, cyclophosphamide : 6 %, mycophenolate : 3 %, ciclosporine : 3 %). Un antecedent de traitement par rituximab etait note chez 21 % des patients avec un delai moyen par rapport a la derniere injection de 1 an (26 j–3 ans), et un nombre moyen d’injections de 4,5. Un patient avait recu un anti-TNF (etanercept) et un autre du tocilizumab mais ils avaient recu d’autres IS depuis. Aucun des patients n’avait d’antecedent de PCP, un seul patient recevait une prophylaxie (pentacarinat). Un taux de lymphocytes CD4 avait ete realise avant l’episode chez 30 % des patients et etait en moyenne de 709/mm 3 (61–2 459). Un taux de lymphocytes CD4 avait ete realise pendant l’episode chez 66 % des patients et etait en moyenne de 333/mm 3 (12–1 202). Il etait > 200/mm 3 dans plus de 70 % des cas. Le taux de lymphocytes totaux, disponible pour tous les patients, etait en moyenne de 946/mm 3 . Plus de 60 % des patients avaient un taux > 600/mm 3 . On denombrait 13 deces au decours immediat de l’episode (40 %) dont 7 directement attribuables a la PCP (21 %). Discussion La PCP chez les patients de medecine interne est une pathologie severe. Les delais de survenue par rapport au diagnostic de la MAI et le debut du traitement IS notamment le rituximab sont tres variables. Une corticotherapie est presque toujours retrouvee a des doses excedant souvent 20 mg/j. Les taux de CD4 et de lymphocytes totaux ne permettent pas de guider l’indication d’une prophylaxie dans le contexte particulier des MAI. Conclusion Une prophylaxie anti-PCP chez les patients de medecine interne doit etre discutee independamment du taux de lymphocytes et avant tout en fonction d’un risque individuel base sur le risque propre lie a la MAI, la presence d’une corticotherapie, et le type d’IS associe.

Introduction La granulomatose eosinophilique avec polyangeite (GEPA) est une vascularite systemique rare, associant le plus souvent une atteinte pulmonaire et ORL avec une hypereosinophilie. Mais elle prend parfois une toute autre presentation. Nous rapportons le cas d’une patiente ayant developpe un syndrome appendiculaire, illustrant les difficultes diagnostiques des vascularites a presentation pleiotrope. Observation Une patiente de 31 ans etait suivie depuis 2012 pour une GEPA selon les criteres de l’ACR, seronegative, avec atteintes cutanee, pulmonaire et articulaire. Le traitement corticoide etait en phase de decroissance avec un passage recent a 7,5 mg/j, sans traitement immunosuppresseur associe. Elle etait hospitalisee en urgence pour une defense febrile en fosse iliaque droite. Les eosinophiles etaient augmentes a 4 500/mm3, soit environ le double de ses valeurs habituelles. Il n’existait pas de syndrome inflammatoire. L’echographie abdominale objectivait un epanchement peri-appendiculaire avec un appendice epaissi, indiquant une prise en charge chirurgicale : l’appendice etait peu inflammatoire mais il existait un epanchement purulent intraperitoneal avec des nodules blanchâtres dans la loge de Morrison. Une appendicectomie et des biopsies des nodules etaient pratiquees et l’analyse anatomopathologique retrouvait des lesions de vascularite necrosante et des micro-abces a eosinophiles. Le traitement etait complete par 2 bolus de methylprednisolone, la reprise d’une corticotherapie orale a 0,7 mg/kg/jour associee a 9 bolus de cyclophosphamide IV toutes les 3 semaines. La patiente a evolue favorablement, permettant la reprise d’un sevrage des corticoides. Discussion Les manifestations respiratoires, ORL et cutanees sont classiques dans la GEPA mais les manifestations digestives ne doivent pas etre negligees. Elles existent dans environ 1/3 des cas, selon les donnees de la litterature [1], et sont liees a une vascularite des petits vaisseaux. Leur pronostic est severe avec notamment un risque de perforation. Nous decrivons un nouveau cas de pseudo-appendicite, evolution rare des GEPA [2,3]. Il pose le probleme du diagnostic differentiel avec les atteintes infectieuses, chez des patients aux tableaux atypiques du fait d’un traitement immunosuppresseur. Une hypereosinophilie inhabituelle pourrait attirer l’attention et orienter le diagnostic. Neanmoins, il semble difficile de se priver du concours du chirurgien pour eliminer de telles atteintes infectieuses, auxquelles ces patients sont frequemment exposes en raison de leur immunosuppression medicamenteuse… Conclusion Les manifestations digestives de la GEPA ne doivent pas etre meconnues. En effet, elles constituent un challenge diagnostique et un critere de gravite de la maladie, justifiant l’introduction urgente d’un traitement immunosuppresseur.

Background Pneumocystis jirovecii pneumonia (PCP) is an opportunistic fungal respiratory infection. The incidence of PCP has decreased among HIV patients, however among non HIV-negative patients on immunosuppressive drugs; an increase in incidence is noted. In this population, the diagnosis of PCP is difficult because the clinical presentation is atypical and the direct examination (DE) of the respiratory secretions is often negative. In this context, detection of Pneumocystis jirovecii DNA in respiratory secretions by real-time quantitative chain reaction (qPCR) should be usefull. Methods In order to evaluate the usefulness of qPCR, all patients hospitalized in medicine or intensive care unit (ICU) in a university hospital and having a positive qPCR in respiratory secretions were included in a retrospective study conducted between 2013 and 2016. Based on clinical data, respiratory secretions, imaging and treatment, patients were classified into three groups: certain PCP, possible, or colonization, irrespective of the value of qPCR. Results One hundred and fifty patients, including 38 infected with HIV, were included: 75 in medicine and 75 in intensive care. Ninety patients (60%) had bronchoalveolar lavage. The diagnosis of PCP was considered certain or possible for 52 and 77 patients respectively and rejected (colonization) for 21 patients. DE was negative for 78% of non-HIV patients and 29% of HIV patients. Among the 129 patients with PCP, the hospital mortality was 35.9% in ICU and 21.5% in medicine. The median value of qPCR was 76,650 copies/mL among patients with PCP and 3,220 copies/mL among colonized patients (P

Introduction Devant une hyperleucocytose majeure associee a des blastes circulants, le diagnostic le plus facilement evoque est celui de leucemie aigue mais des diagnostics differentiels sont a envisager. Observation Un patient de 55 ans etait adresse aux urgences pour une hyperleucocytose a plus de 500 000/mm3. Ce bilan avait ete realise dans un contexte d’asthenie evoluant depuis 3 semaines associee a une douleur permanente de l’hypochondre gauche. Il n’avait pas d’antecedent personnel ou familial. Sur la biologie initiale, la creatinine etait a 97 μmol/L, l’acide urique a 697 μmol/L, les LDH a 1519 UI/L ; temoin d’une lyse spontanee. L’hemoglobine etait a 10,5 g/dL, le VGM a 106, les plaquettes a 227 000/mm3, les leucocytes a 594 000/mm3 sans anomalie de la coagulation. Une volumineuse hepato-splenomegalie etait palpable a 4 travers de doigt, ainsi que des adenopathies inguinales inferieures a 2 cm, sans atteinte cutaneo-muqueuse. Il n’y avait pas d’argument pour une leucostase. Les frottis medullaire et sanguin en urgence mettaient en evidence une population heterogene de cellules plus ou moins immatures de taille variable, de haut rapport nucleo-cytoplasmique, a chromatine un peu lâche, et noyaux generalement ronds, mais parfois irreguliers, voire foliaces. Par ailleurs, on notait la presence de cellules avec une chromatine plus fine, nucleolee. Le cytoplasme etait basophile, non granuleux. Le test a la peroxydase sur sang et mœlle etait negatif. Les serologies etaient negatives. Un scanner thoraco-abdomino-pelvien permettait de visualiser une volumineuse hepato-splenomegalie. Il existait des hypodensites spleniques en faveur d’infarctus recents probablement responsable du tableau douloureux initial. Un diagnostic de lymphome du manteau de forme blastoide etait propose sur les aspects cytologiques et confirme par l’immunophenotypage sanguin et medullaire en faveur d’une hemopathie lymphoide B kappa CD5+/CD23–/CD20+/CD10–ainsi que sur la biopsie medullaire avec un infiltrat massif (80 %) CD20+/CD5+/CD3–/CD10–/CD23–avec Bcl2 et cycline D-1 positif. Une chimiotherapie par cyclophosphamide, vincristine et prednisone etait debutee avec une diminution rapide des cellules lymphomateuses circulantes, une autogreffe etait envisage apres chimiotherapie par rituximab, aracytine, carboplatine, dexamethasone. Discussion Les lymphomes du manteau sont une entite rare (7–9 % des lymphomes) survenant generalement chez des individus de plus de 60 ans avec une nette predominance masculine. La presentation est souvent d’emblee generalisee et agressive avec une polyadenopathie, une splenomegalie et une atteinte medullaire. Le pronostic reste sombre, lie principalement aux rechutes frequentes et aux stades avances au diagnostic [1] . Dans 20–30 % des cas, il existe des cellules lymphomateuses circulantes. Le phenotype est generalement celui d’une cellule B mature (CD5+ CD23–) avec une sur-expression de la cycline D1. Ce dernier element est caracteristique des lymphomes du manteau et resulte d’une translocation t(11 ; 14) (q13 ; q32) permettant la juxtaposition du gene codant pour les chaines lourdes d’immunoglobuline dans la region promotrice de la cycline D1, entrainant son expression constitutive et une deregulation du cycle cellulaire. Quatre formes sont distinguees au sein des lymphomes du manteau en fonction de l’aspect en cytologie [2] : la forme a petites cellules, la forme classique du manteau, la forme pleiomorphe, et la forme blastique ou blastoide. Cette derniere consideree comme la plus agressive et dont les cellules ressemblent a des lymphoblastes possedent une chromatine lâche et un index mitotique eleve. Cette forme peut donc etre confondue avec une leucemie aigue lymphobastique (LAL), ou une leucemie a prolymphocytes B. Conclusion La presentation blastique d’emblee et la tres haute cellularite contrastent malgre tout dans notre cas clinique avec un etat general moderement altere et surtout une absence de leucostase ainsi qu’une lyse spontanee modeste. La splenomegalie majeure initiale peut egalement faire evoquer des diagnostics alternatifs comme : une leucemie a tricholeucocytes (toutefois absence de monocytopenie dans notre cas), un lymphome splenique a lymphocytes villeux ou une leucemie lymphoide chronique. C’est l’immunophenotypage sanguin et medullaire qui a permis de poser avec certitude le diagnostic de lymphome du manteau de forme blastoide. L’interet de cette observation reside dans l’importance de l’hyperleucocytose pouvant egarer le diagnostic vers une leucemie aigue ; rares sont les cas rapportes de lymphome du manteau avec une hyperleucocytose aussi majeure (2 cas) [3] .