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Les 3 principaux types d'amylose cardiaque sont la conséquence de dépôts organiques de deux précurseurs protéiques : d'une part l'amylose AL (AL), secondaire à des dépôts de chaînes légères d'immunoglobulines dans le cadre d'une gammapathie monoclonale (essentiellement de signification indéterminée ou d'un myélome), et d'autre part les amyloses à transthyrétine (ATTR), comprenant les amyloses à transthyrétine sauvages et les amyloses à transthyrétine héréditaires. La fréquence de ces maladies est probablement sous-estimée, comme le montrent les études récentes sur sa prévalence élevée dans des phénotypes cardiologiques courants (insuffisance cardiaque à fraction d'éjection préservée, rétrécissement aortique serré, cardiomyopathie hypertrophique). L'infiltration amyloïde myocardique concerne toutes les structures cardiaques et peut conduire à la survenue d'une insuffisance cardiaque, de troubles conductifs et d'évènements cardio-emboliques. La recherche des signes extracardiaques est une aide au diagnostic. L'électrocardiogramme, l'échocardiographie et l'IRM cardiaque permettent de suspecter l'amylose cardiaque. La confirmation diagnostique se fait sur l'application d'un algorithme simple incluant une recherche systématique de gammapathie monoclonale par un bilan protéique complet et une scintigraphie aux disphosphonates (traceur osseux DPD, HMDP, PyP). Une documentation histologique est impérative en cas d'AL ; elle sera également requise en cas d'ATTR s'il existe une immunoglobuline monoclonale et/ou un ratio anormal de chaînes légères afin d'initier le traitement spécifique. Compte tenu de l'âge tardif d'apparition de la maladie dans les formes héréditaires, la réalisation d'un test génétique est systématique en cas d'ATTR. Ces maladies n'étant plus perçues comme incurables depuis les récentes innovations thérapeutiques, une meilleure (re)connaissance de la maladie est plus que jamais nécessaire. The 3 main types of cardiac amyloidosis are linked to two protein precursors: AL amyloidosis secondary to free light chain deposits in the context of monoclonal gammopathy (mainly of undetermined significance or myeloma) and transthyretin amyloidosis (ATTR), comprising wild-type transthyretin amyloidosis (ATTRwt for wild type) and hereditary transthyretin amyloidosis (ATTRv for variant). These diseases are underdiagnosed and highly prevalent in common cardiac phenotypes in recent studies (heart failure with preserved ejection fraction, severe aortic stenosis, hypertrophic cardiomyopathy). Myocardial amyloid infiltration affects all cardiac structures and clinically promotes predominantly heart failure, conductive disorders and cardioembolic events. The search for extracardiac signs makes it possible to arouse diagnostic suspicion. Electrocardiogram, echocardiography and cardiac MRI can suspect cardiac amyloidosis. The diagnostic confirmation follows a simple algorithm including a systematic search for monoclonal gammapathy and a disphosphonate scintigraphy. Histological proof is necessary in case of AL or ATTR amyloidosis with concomitant monoclonal gammopathy in order to initiate specific treatment. Due to the late disease onset in ATTRv, genetic testing must be routine in all cases of ATTR. These diseases are no longer perceived as incurable since recent therapeutic innovations. A better knowledge of the disease is more than ever necessary. [ABSTRACT FROM AUTHOR]

Background: Central nervous system (CNS) relapse is an uncommon but dramatic complication of diffuse large B-cell lymphoma (DLBCL). Several studies have demonstrated the superiority of cerebrospinal fluid (CSF) flow cytometry (FCM), as compared with conventional cytology (CC), in detecting occult leptomeningeal disease. The clinical relevance of a positive FCM still has to be clarified. Patients and methods: We analyzed CSF from 114 DLBCL patients at diagnosis (n = 95) or at relapse (n = 19) by FCM and CC. Most patients received meningeal prophylaxis. FCM results did not influence treatment strategies. Results: Fourteen samples were FCM+, versus one CC+ (also FCM+). Within all patients without neurological symptoms (n = 101), four (4%) relapsed in the CNS, with a median time to relapse of 5.2 months. Only one-fourth (25%) was FCM+ before relapse. More than one extranodal disease site and elevated lactate dehydrogenase levels were associated with an increased risk of CNS relapse. Conclusions: FCM gives far more positive results than CC. However, a positive FCM result did not translate into a significant increase in CNS relapse rate in this histologically uniform population receiving CNS prophylaxis. [ABSTRACT FROM PUBLISHER]

Abstract: The electrophysiological evaluation of Wolff–Parkinson–White syndrome (WPW) is recommended in children aged more than five years to detect a risk of life-threatening arrhythmia. The purposes of the study were to determine the feasibility of transesophageal EPS in a child between six and 10 years in out-patient clinic. Methods: Electrophysiological study (EPS) was indicated in 22 children, aged six to 10 years, with a manifest WPW either for no documented tachycardia (n =7), unexplained dizziness (n =2) or for a sportive authorization in 10 asymptomatic children. Two of the last children had a history of permanent tachycardia after the birth but were asymptomatic since the age of one year without drugs. Results: EPS was performed in all children. The main difficulty lied in passing the catheter through the mouth. Programmed stimulation at cycle length of 380ms was performed in all children to avoid high rates of pacing when the conduction through the accessory pathway (AP) and normal AV system was evaluated. Isoproterenol was not required in five children, because they developed a catecholaminergic sinus tachycardia. The AP refractory period was determined in all children between 200 and 270ms. Orthodromic reentrant tachycardia (RT) was induced in 11 children, three asymptomatic children (27%), seven complaining of tachycardia and one with syncope. Rapid antidromic tachycardia was induced in this last child with dizziness. Atrial fibrillation was never induced. Conclusions: Esophageal EPS can be performed without sedation in a young child six to 10-year-old with a shortened protocol of stimulation, which was capable to clearly evaluate the WPW-related risks. [Copyright &y& Elsevier]