Characteristics of large granular lymphocyte leukemia associated with variable common immunodeficiency disorders: A study of 12 cases.

OBJECTIVES: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL. METHODOLOGY: Patients were included through a call for comments to French and Belgian centers and through a literature review via PubMed. Clinical characteristics were compared to two large French cohort involving CVID and LGLL patients. RESULTS: Twelve patients were included. In all cases, CVID precedes LLGL (median diagnosis delay for LLGL was 7 years). Most cases presented with splenomegaly and autoimmune cytopenia. Ten out of 12 patients underwent splenectomy during follow up. CONCLUSIONS: Patients with LGLL and CVID differ from patients without immune deficiency in term of clinical presentation and prognosis. We suggest CVID may act as a trigger of LGL lymphocytosis, due to endogenous and exogenous antigenic pressure leading to the selection of a dominant LGL clone and stimulation of the JAK-STAT pathway. The role of splenomegaly and splenectomy in LGLL onset warrant further investigation in future studies.

Management of infective endocarditis and multidisciplinary approach.

INTRODUCTION: The morbi-mortality related to infective endocarditis (IE) remains high as the epidemiology has changed over the last years: ageing of patients, comorbidity and healthcare-associated infections. To optimize IE management, a weekly endocarditis multidisciplinary meeting (EMM) was set up at our facility. We present the activity report of the EMM. PATIENTS AND METHODS: All patients hospitalized for IE who were presented at the weekly EMM between January 2013 and June 2017 were prospectively included. The main objective was to assess the impact of the EMM on the management of community-acquired IE and healthcare-associated IE by analyzing in-hospital case fatality. RESULTS: Of the 1139 cases reported during the EMM for suspicion of IE, 493 (86% were definite cases) were selected for the study: 262 patients had community-acquired IE and 231 had healthcare-associated IE; 43% of IEs involved a valvular prosthesis. Following the EMM, infections were documented in 92% of cases: staphylococci in 45% of healthcare-associated IEs and streptococci in 44% of community-acquired IE cases. A septic embolism was diagnosed in 57% of cases. Finally, 49% of patients underwent surgery. The in-hospital case fatality was 12% with no significant difference between community-acquired IEs and healthcare-associated IEs. Case fatality was also significantly higher in elderly patients, in the absence of surgical treatment, initial heart failure, or Staphylococcus aureus IE. CONCLUSION: The weekly EMM allows our facility to follow the European Society of Cardiology guidelines and to adapt the management of each patient to improve IE prognosis.

[Trochanteric bursitis, pelvic enthesopathy and giant cell arteritis]. / Bursite trochantérienne, enthésopathie pelvienne et maladie de Horton.

Giant cell arteritis, a large-sized vessel vasculitis, may be associated with musculoskeletal proximal (polymyalgia rheumatica) or distal manifestations. A 68-year-old woman, who had inflammatory pelvic girdle pain, was diagnosed with giant cell arteritis and was successfully treated with corticosteroids. The magnetic resonance imaging and ultrasonography revealed a bilateral bursitis and pelvic girdle enthesopathy. Bursitis is the main anatomic lesion occurring in polymyalgia rheumatica and can be underlined by ultrasonography.

Graft infection after a Bentall procedure: A case series and systematic review of the literature.

INTRODUCTION: The Bentall procedure is a cardiac surgery involving graft replacement of the aortic valve, aortic root and ascending aorta. Graft infection after Bentall's procedure (BGI) is infrequent but severe, and often difficult to diagnose and treat. PATIENTS AND METHODS: A retrospective cohort study was performed using the Bordeaux endocarditis database of adult patients admitted to the Bordeaux University Medical Hospital for BGI between 2008 and 2014. Published case reports were identified in the literature. RESULTS: We identified 10 BGI patients in the database and 13 in the literature. The majority of infections were late-onset (20/23) and occurred as a result of gram positive cocci bacterial infection (16/22). Detailed diagnoses of the described BGI were determined using echocardiography, computed tomography (CT) and positron emission tomography/CT (PET/CT). Labeled-leukocyte scintigraphy was not reported in any case. Prolonged antibiotic therapy and surgery were found to be the treatment of choice for BGI; however it was not always possible to perform a surgical intervention. Clinical relapses occurred even with a negative PET/CT, while PET/CT consistently positive for BGI occurred in the absence of clinical relapse. This suggests that the use of PET/CT for follow-up is questionable. CONCLUSION: Diagnosis of BGI is difficult, due to the combination of clinical, biological, and radiological observations obtained through transesophageal echocardiography and CT. PET/CT is an alternative method to diagnosis BGI, but its impact on clinical management remains unclear. Current data suggests that if surgical replacement of the prosthesis is not possible, patients should be treated with prolonged antibiotic therapy.

Infective endocarditis caused by Streptococcus tigurinus-like organisms.

Streptococcus species are important causes of infective endocarditis but species identification remains challenging. We report two cases of infective endocarditis due to Streptococcus tigurinus-like organisms, which were first identified by 16S ribosomal RNA gene sequence analysis and subsequently confirmed using phylogeny based on the analysis of the shetA gene encoding exfoliative toxin.

[Hydrocycarbamide induced fever: four cases and literature review]. / Hyperthermie induite par l'hydroxyurée : à propos de 4 cas et revue de la littérature.

PURPOSE: Hydroxyurea (HU) or hydroxycarbamide is an antimetabolite chemotherapy frequently used in the treatment of chronic myeloproliferative disorders. This treatment is usually well tolerated but a few cases of fever induced by the molecule have been reported in the literature. The aim of the study was to describe the clinical and biological characteristics of HU induced fever. METHODS: We performed a cross sectional study of patients treated with HU and followed-up in an internal medicine department between 2006 and 2012. We added our cases of HU induced fever with those reported in the literature (Pubmed and Cochrane databases) since 1981. RESULTS: We identified 38 cases of HU induced fever, including our 4 cases. The mean age was 65±10.9 years and the sex-ratio 1/2. The fever appeared after a median duration of treatment of 21 days and was usually high (40°C) but clinically well tolerated. A biological inflammatory syndrome (CRP: 131±92 mg/L) was constant and one third of the patients also presented with hepatitis or lung disease. A probabilistic antibiotic treatment was introduced for 34% of the patients. For the half of the patients, HU-reintroduction test was performed, and was positive for all the patients but one. As soon as HU was withdrawn, the fever disappeared in a median of 24 hours. CONCLUSION: HU induced fever is unusual. Clinical presentation is very stereotyped. When this adverse effect is suspected, an infectious disease must first be ruled out. If infection is excluded, HU has to be stopped.

[Large granular lymphocyte leukemia: clinical and pathogenic aspects]. / Les leucémies à grands lymphocytes granuleux : de la clinique à la physiopathologie.

Large granular lymphocyte leukemia (LGL) is a hematologic disorder characterized by a monoclonal expansion of large lymphocytes containing azurophilic granules with a T CD3(+)CD57(+) or Natural Killer (NK) CD3(-)CD56(+) phenotype. The World Health Organization (WHO) classification identifies three entities: the T LGL, the chronic lymphoproliferative disorder of NK-cells, and the aggressive NK-cell leukemia. T LGL and chronic lymphoproliferative disorder of NK-cells are indolent diseases frequently associated with cytopenias and a wide spectrum of auto-immune manifestations. Neutropenia can lead to recurrent bacterial infections, which represent an indication of initiating a treatment in most of the cases. Immunosuppressive therapies are usually used in this context. In contrast, aggressive NK-cell leukemia follows a fulminant course with a poor prognosis because patients are refractory to most of the treatments. There is now a considerable interest in the pathophysiology of the disease with the perspective of new therapeutic options.

Reactive arthritis associated with Mycoplasma genitalium urethritis.

Mycoplasma genitalium is an important cause of sexually transmitted infections that is gaining recognition and is an independent cause of acute and chronic nongonococcal urethritis in men. M. genitalium has been implicated as a possible causative factor in reactive arthritis. We report a case of reactive arthritis complicating M. genitalium urethritis in an HLA-B27-positive patient.

[Intravascular large B cell lymphoma: a case series of three patients and update]. / Lymphome à grandes cellules B intravasculaire : trois cas et mise au point.

PURPOSE: Intravascular large B cell lymphoma (IVLBCL) is a rare and aggressive variant of non-Hodgkin's lymphoma, characterized by multifocal proliferation of lymphoma cells exclusively within small and medium blood vessels. IVLBCL can be systemic and quite polymorphic, which often makes it difficult to diagnose, thus delaying appropriate treatment. PATIENTS AND METHODS: We report three patients of atypical IVLBCL, through the study of which we overview recent knowledge about IVLBCL. RESULTS: The first patient initially presented with peripheral thrombocytopenia and splenic destruction of platelets, later completed with an interstitial pulmonary syndrome. The second patient, of African origin, we believe is the first case of a black patient with IVLBCL described in the medical literature. The third belongs to the rare group of occidental patients that present an IVLBCL associated with a hemophagocytic syndrome. CONCLUSION: Intravascular large B cell lymphoma is a systemic and polymorphic disease. Awareness of this entitiy should allow rapid and appropriate management.

[Continuous acoustic monitoring of nocturnal bronchial obstructions]. / Die akustische Langzeitregistrierung nächtlicher Bronchialobstruktionen.

Patients with bronchial asthma often have respiratory problems in sleep. The effects of bronchial obstructions while sleeping have been analysed in some studies. For an exact assessment the sleep itself must not be disturbed by the method. The continuous acoustic lung sound detection is such a method. It helps to assess the circadian rhythm during antiobstructive therapy which may lead to a better sleep quality and daytime fitness.