Syndrome des antisynthétases et adénocarcinome pulmonaire : une association fortuite ?

Introduction

Dermatomyositis and polymyositis are sometimes associated with neoplasia. Conversely, a link between antisynthetase syndrome and neoplasia has not been clearly demonstrated.

Case report

We report a 54-year-old smoker male patient who presented with an antisynthetase syndrome with anti-Jo1 and anti-Ro-52 antibodies. An adenocarcinoma of the lung was diagnosed at the same time.

Conclusion

Two recent studies showed that patients with an antisynthetase syndrome associated with anti-Jo1 antibodies have more severe prognosis than antisynthetase syndrome associated with other antibodies (i.e. PL7/PL12). The risk of cancer occurrence seems to be increased when the anti-Jo1 antisynthetase syndrome is associated with anti-Ro-52 antibodies. To date, there is no demonstrated association between antisynthetase syndrome and neoplasia.     

La bronchopneumopathie chronique obstructive : une maladie auto-immune ?

Chronic obstructive pulmonary disease (COPD) is an important cause of morbidity and mortality characterized by irreversible airflow limitation involving a reduced caliber of distal airways (less than 2mm) and alveolar destruction. Exposure to tobacco is a major risk factor for COPD, but all smokers do not develop the disease. In addition, there is continued progression of the disease several years after cessation of the exposure. To explain these phenomena, factors involving innate immunity including the release of neutrophil elastase, macrophage metalloproteases, in combination with pro-apoptotic factors, involved in the worsening of the lesions of emphysema and fibrosis of small airways have been described for many years. More recently, it has been proposed at an advanced stage of the disease that an autoimmune reaction directed mainly at elastin could participate to the pathogenesis of the disease. We here review the immunological processes and currently available data on autoimmunity in COPD.     

Fibrose rétropéritonéale et myélome multiple : association fortuite ?

We report a 59-year-old man presenting with retroperitoneal fibrosis (RF) associated with IgG lambda multiple myeloma. Recent clinical and immunohistochemical findings suggest that RF might be a particular expression of plasma cell/lymphoid dyscrasia, and that this association is not merely fortuitous. We review the pathophysiological evidence supporting this hypothesis.     

Syndrome TAFRO et vascularite nécrosante cutanée : une association inédite

IntroductionTAFRO syndrome is a systemic inflammatory syndrome in the spectrum of Castleman's disease, associating thrombocytopenia, anasarca, fever, renal failure and/or reticulin myelofibrosis and organomegaly. Its association with necrotizing cutaneous vasculitis has not yet been reported.Case reportA 69-year-old woman presented with weight loss, fever, anasarca, organomegaly, lymphadenopathy, anuria and extensive necrotic livedo occurring after acute diarrhea. Biology showed anemia, thrombocytopenia, renal failure, hypergammaglobulinemia, a circulating B-lymphocyte clone, hypoparathyroidism and autoimmune hypothyroidism. The skin biopsy showed small vessel vasculitis with fibrinoid necrosis. Methylprednisolone infusions associated with tocilizumab were ineffective and the patient became anuric. Rituximab and plasma exchanges associated to corticosteroids allowed remission for 2 months. Combination of rituximab, cyclophosphamide and dexamethasone resulted in a prolonged remission.ConclusionWe report here the first case of severe cutaneous necrotizing vasculitis in a patient suffering from TAFRO syndrome. The possible resistance to tocilizumab should be known.     

Syndrome de Guillain-Barré : physiopathologie et aspects diagnostiques

Guillain-Barré syndrome (GBS) is the most common cause of acute neuropathy. It usually onset with a rapidly progressive ascending bilateral weakness with sensory disturbances, and patients may require intensive treatment and close monitoring as about 30% have a respiratory muscle weakness and about 10% have autonomic dysfunction. The diagnosis of GBS is based on clinical history and examination. Complementary examinations are performed to rule out a differential diagnosis and to secondarily confirm the diagnosis. GBS is usually preceded by an infectious event in ≈ 2/3 of cases. Infection leads to an immune response directed against carbohydrate antigens located on the infectious agent and the formation of anti-ganglioside antibodies. By molecular mimicry, these antibodies can target structurally similar carbohydrates found on host's nerves. Their binding results in nerve conduction failure or/and demyelination which can lead to axonal loss. Some anti-ganglioside antibodies are associated with particular variants of GBS: the Miller-Fisher syndrome, facial diplegia and paresthesias, the pharyngo-cervico-brachial variant, the paraparetic variant, and the Bickerstaff brainstem encephalitis. Their semiological differences might be explained by a distinct expression of gangliosides among nerves. The aim of this review is to present pathophysiological aspects and the diagnostic approach of GBS and its variants.     

Transplantation hépatique de l’adulte: qui et quand greffer?

Introduction. -- The shortage of cadaveric organ donors imposes a severe limit to the number of liver transplantations. A selection is thus necessary among patients: should the sickest be selected, or those who supposedly have the best chance to survive and recover? Optimizing the timing of transplantation during the course of the disease (not too early, but not too late) is another issue.Current knowledge and key points. -- Suitable candidates for transplantation are patients suffering from an irreversible, symptomatic liver disease. The goals of therapy are: firstly, to favorably modify the natural outcome of the disease; and secondly, in an acceptable risk taking manner. Major criterias for indication in the most common liver diseases can be summerized as follows: a) for chronic parenchymal liver diseases, a Child-Pugh score of 9 or 10, or less if complications have already occurred, is a mandatory and often sufficient criterion; b) for cholestatic liver diseases, a serum bilirubin level higher than 100-150 μmol/L is generally required; c) apart from «small» hepatocellular carcinomas on cirrhotic parenchyma (less than three tumors of less than 5 cm in diameter), most cancers are considered contraindications; d) acute liver failure requires early referral to a liver transplant center for potential emergency indication.Future prospects and projects. -- In an organ shortage situation which is likely to perdure, early consultative contact between the patient and the liver transplant team will allow improvement in the access to transplantation procedure.

Résumé

Introduction. -- L'augmentation du nombre de transplantations hépatiques est limitée par l'insuffisance des ressources en donneurs. La nécessité impose donc un choix parmi les patients susceptibles de bénéficier de ce traitement -- les cas plus graves? ou ceux qui ont les meilleures chances de guérir? -- et une évaluation optimale du moment de la transplantation dans l'histoire de la maladie: ni trop tôt, ni trop tard.Actualités et points forts. -- La transplantation doit être réservée aux patients souffrant d'une affection hépatique irréversible et symptomatique. Elle doit, en règle générale et par ordre d'importance, d'une part modifier l'histoire naturelle de la maladie et, d'autre part, pour un risque minimal. Les critères majeurs d'indication peuvent être ainsi schématisés pour les hépatopathies les plus fréquentes: 1) pour les cirrhoses non choléstatiques, un score de Pugh-Child de 9-10, ou un score inférieur si des accidents évolutifs se sont déjà produits, est un critère nécessaire et souvent suffisant ; 2) pour les cirrhoses choléstatiques, un taux de bilirubine sérique supérieur à 100-150 μmol/L est un critère admis ; 3) si l'on excepte les «petits» carcinomes hépatocellulaires sur cirrhose (moins de trois nodules de moins de 5 cm), presque tous les cancers sont une contre-indication à la transplantation ; 4) l'insuffisance hépatique aiguë nécessite un transfert précoce en milieu spécialisé pour une éventuelle indication de transplantation en urgence.Perspectives et projets. -- Dans un contexte de pénurie qui risque de perdurer, l'amélioration des conditions d'accès à la transplantation nécessite qu'un avis consultatif puisse être pris précocement dans l'histoire naturelle de la maladie auprès des équipes en charge de la transplantation.     

Radiothérapie externe et photothérapie dynamique des cancers de l’œsophage : une association dangereuse ?

A 60-year-old man presented an oesophageal transmural necrosis fistulised in the trachea following curative photodynamic therapy (PDT) for a superficial recurrence of an oesophageal carcinoma, initially treated by radiochemotherapy. Two stents, a tracheal and an oesophageal one, were placed. Eight months later the patient is in complete remission with only mild swallowing problems. This complication, although unusual, has already been described by other teams with the association of radiochemotherapy and PDT. The present case study suggests that illumination dose should be lowered in this indication.     

Inhibiteurs de la pompe à protons et clopidogrel (Plavix®) : une liaison dangereuse ?

Proton pump inhibitors (PPI) and antiplatelet agents, especially aspirin and clopidogrel, are among the most prescribed medications worldwide. Their co-administration is justified by the increased risk of gastrointestinal bleeding related to the antiplatelet therapy. The issue of the interaction between PPI and clopidogrel has been raised with the emergence of the concept of “high on-clopidogrel platelet reactivity” (or “clopidogrel resistance”) together with the discovery of the role of CYP2C19 isoform in the pharmacokinetics of those two medications. Indeed, CYP2C19 is involved in the conversion of the clopidogrel pro-drug into its active metabolite and is involved in the metabolisation of PPI into inactive metabolites, acting as substrates/inhibitors of CYP2C19. Despite their heterogeneity, most pharmacodynamic studies have shown a decreased clopidogrel antiplatelet effect when associated to PPI, especially those with the highest CYP2C19 inhibiting activity (omeprazole, lansoprazole, rabeprazole). On the other hand, clinical studies are inconclusive. Retrospective studies have shown an increased risk of major cardiovascular events or mortality when clopidogrel and PPI are associated in comparison with clopidogrel alone, particularly in the patients with the higher cardiovascular risk. However, the two prospective randomized studies published so far did not find any interaction and confirmed the benefit of PPI on the gastrointestinal bleeding. As a conclusion, as the clinical studies are not conclusive, the French health authorities have recently removed the alert about this interaction. PPI and clopidogrel can thus be co-prescribed.     

Histiocytose langerhansienne et maladie d’Erdheim-Chester,une continuité ?

IntroductionErdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described.ObservationA 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation. The patient was treated with vemurafenib with a good clinical course.ConclusionThe literature review finds forty observations linking the two diseases that may suggest a pathophysiological link, especially with the hematopoietic myeloid stem cell CD34+. The term inflammatory myeloid neoplasm was advanced.     

Plasmocytose sanguine et dengue : une anomalie biologique sous-estimée ?

Introduction

A significant peripheral blood plasmacytosis is a rare finding associated with viral infections. We reported five consecutive cases of dengue virus infection, with circulating plasma cells.

Case reports

Three women and two men, aged 26 to 75 years, had returned from French West Indies less than one week before the onset of the symptoms (mean: 2.5 days). The transient blood plasmacytosis was variable in intensity (0.1 to 0.8 G/L) with a maximal level between the fourth and the seventh day following the onset of the symptoms, and was associated in four patients, with activated lymphocytes and lympho-plasma cells.

Conclusion

Reactive plasmacytosis during dengue fever is common and probably underestimated because it is transient and only identified by careful microscopic examination of a blood smear. Plasmacytosis could be explained by the intensity of the immunological response and the production of large amount of interleukins.