Erdheim-Chester disease: a rare histiocytosis (case report and review of the literature)

We report a case of Erdheim-chester disease, a rare non-langerhans histiocytosis revealed by polydipsia-polyuria syndrome in a 26 years old woman, trated by interferon with a good response

Complications neurologiques de la carence en thiamine (Vitamine B1) : A propos d’un cas avec revue de la litterature

Introduction: La carence en thiamine peut se manifester par deux formes cliniques : une forme cardiaque nommée béribéri humide, et une forme neurologique qui est le béribéri sec ; cette dernière peut se traduire cliniquement par une neuropathie périphérique, ou par une encéphalopathie de Gayet-Wernicke, caractérisée par la triade classique : ophtalmoplégie, ataxie et état confusionnel. Cette encéphalopathie est souvent difficile à identifier et son diagnostic est retardé. Observation: Nous rapportons le cas d’une patiente de 27 ans, suivie pour leucémie aigue promyélocytaire réfractaire, avec atteinte du système nerveux central, ayant eu une alimentation parentérale prolongée, en raison des vomissements incoercibles et persistants, suite à laquelle elle a développé une encéphalopathie de Wernicke, ayant bien évolué sous supplémentation vitaminique intra-veineuse. Commentaires:Nous aborderons dans ce travail, l’intérêt du diagnostic précoce, les différents contextes évocateurs et les aspects clinique, radiologique ainsi que les options thérapeutiques dans l’encéphalopathie de Wernicke.   English title: Neurological Complications Of Thiamine Deficiency, A Case Report With Literature Review Background: Thiamine deficiency can manifest itself in two clinical forms: a cardiac form called wet beriberi, and a neurological form which is dry beriberi; the latter can result clinically by peripheral neuropathy, or by Gayet-Wernicke encephalopathy, characterized by the classic triad: ophthalmoplegia, ataxia and confusional state. This encephalopathy is often difficult to identify and its diagnosis is delayed. Observation: We report the case of a 27-year-old patient, followed for refractory acute promyelocytic leukemia, with central nervous system damage, having had a prolonged parenteral diet, due to incoercible and persistent vomiting, following which she developed an encephalopathy of Wernicke, having evolved well under intravenous vitamin supplementation. Comments: In this work, we will discuss the interest of early diagnosis, the different suggestive contexts and the clinical, radiological aspects as well as the therapeutic options in Wernicke's encephalopathy

The evolving SARS-CoV-2 epidemic in Africa: Insights from rapidly expanding genomic surveillance

INTRODUCTION Investment in Africa over the past year with regard to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) sequencing has led to a massive increase in the number of sequences, which, to date, exceeds 100,000 sequences generated to track the pandemic on the continent. These sequences have profoundly affected how public health officials in Africa have navigated the COVID-19 pandemic. RATIONALE We demonstrate how the first 100,000 SARS-CoV-2 sequences from Africa have helped monitor the epidemic on the continent, how genomic surveillance expanded over the course of the pandemic, and how we adapted our sequencing methods to deal with an evolving virus. Finally, we also examine how viral lineages have spread across the continent in a phylogeographic framework to gain insights into the underlying temporal and spatial transmission dynamics for several variants of concern (VOCs). RESULTS Our results indicate that the number of countries in Africa that can sequence the virus within their own borders is growing and that this is coupled with a shorter turnaround time from the time of sampling to sequence submission. Ongoing evolution necessitated the continual updating of primer sets, and, as a result, eight primer sets were designed in tandem with viral evolution and used to ensure effective sequencing of the virus. The pandemic unfolded through multiple waves of infection that were each driven by distinct genetic lineages, with B.1-like ancestral strains associated with the first pandemic wave of infections in 2020. Successive waves on the continent were fueled by different VOCs, with Alpha and Beta cocirculating in distinct spatial patterns during the second wave and Delta and Omicron affecting the whole continent during the third and fourth waves, respectively. Phylogeographic reconstruction points toward distinct differences in viral importation and exportation patterns associated with the Alpha, Beta, Delta, and Omicron variants and subvariants, when considering both Africa versus the rest of the world and viral dissemination within the continent. Our epidemiological and phylogenetic inferences therefore underscore the heterogeneous nature of the pandemic on the continent and highlight key insights and challenges, for instance, recognizing the limitations of low testing proportions. We also highlight the early warning capacity that genomic surveillance in Africa has had for the rest of the world with the detection of new lineages and variants, the most recent being the characterization of various Omicron subvariants. CONCLUSION Sustained investment for diagnostics and genomic surveillance in Africa is needed as the virus continues to evolve. This is important not only to help combat SARS-CoV-2 on the continent but also because it can be used as a platform to help address the many emerging and reemerging infectious disease threats in Africa. In particular, capacity building for local sequencing within countries or within the continent should be prioritized because this is generally associated with shorter turnaround times, providing the most benefit to local public health authorities tasked with pandemic response and mitigation and allowing for the fastest reaction to localized outbreaks. These investments are crucial for pandemic preparedness and response and will serve the health of the continent well into the 21st century

Traitement des amyloses AL systémiques: à propos de 25 cas

L'amylose AL systémique primitive est un désordre hématologique rare. La plupart des recommandations thérapeutiques sont basées sur des études de phase II ou des comparaisons rétrospectives et des séries de cas. Le but de cette étude était de décrire les cas d'amylose primitive AL et de faire une comparaison entre le protocole standard Melphlan-Dexamethasone et les nouveaux agents dans le traitement de première ligne de ces patients. Il s'agissait d'une étude rétrospective, descriptive et multicentrique, portant sur l'ensemble des cas d'amyloses AL colligées durant une période s'étalant de juillet 2009 à juin 2016 au sein de 2 centres hospitaliers militaires. Vingt cinq patients ont été colligés dans notre série (12 traités par le Melphalan-Dexamethasone et 13 par des protocoles contenant au moins du Bortézomib ou du Lénalidomide). Il n'y avait pas de différence significative entre les 2 groupes en termes de caractéristiques épidémiologiques, cliniques ou pronostiques. Après un suivi médian de 40 mois, la survie globale médiane était de 54 mois dans le groupe melphalan-Dexamethasone et de 60 mois dans le groupe nouvelles thérapeutiques (P = 0,98). Concernant la survie sans progression, elle était de 18 mois pour le groupe traitement standard contre 11 mois pour le 2ème groupe (P = 0,08). Dans notre petite série nous n'avons pas trouvé une supériorité des nouvelles thérapeutiques par rapport au protocole classique. Ce résultat doit être confirmé par la réalisation d'une vraie étude prospective surtout en raison du coûtde ces nouvelles molécules qui ne sont pas toujours accessibles surtout dans les pays en voie de développement.Mots clés: Amylose AL, Melphan-Dexamethasone, nouveau traitementEnglish Title: Treatment of systemic AL amyloidosis: about 25 casesEnglish AbstractPrimarye systemic AL amyloidosis is a rare hematologic disorder. The majority of the therapeutic guidelines are based on phase II studies or on retrospective comparisons and case series. Our study aimed to describe all the cases of primary AL amyloidosis reported in 2 military hospitals and to make a comparison between standard melphalan-dexamethasone protocol and new agents in first-line treatment of patients with this disease. We conducted a retrospective, descriptive and multicentric study of all patients with AL amyloidosis whose data were collected during the period July 2009-June 2016. Twenty five patients were enrolled in the study (12 patients treated with melphalan-dexamethasone and 13 with bortezomib-based protocol or lenalidomide-based protocol). There was no significant difference in the epidemiological, clinical and prognostic features between the 2 groups. After a median follow up of 40 months, median overall survival was 54 months in the melphalan-dexamethasone-treated group and 60 months in the new therapies-treated group (P = 0.98). Progression-free survival was 18 months in the standard treatment group vs 11 months in the 2nd group (p = 0.08). In our small case series we haven’t found a superiority of the new therapies compared to the standard protocol. This result should be confirmed by a true prospective study, mainly because of the cost of these new molecules that are not always accessible, especially in developing countries.Keywords: AL amyloidosis, melphalan-dexamethasone, new treatmen

Une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome natural killer nasal

Nous rapportons un cas unique d´une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome non hodgkinien T de type natural killer nasal

Acute Laryngeal Dyspnea as the First Presentation of Mantle Cell Lymphoma: A Case Report and Review of the Literature

Introduction. Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. Case Presentation. We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free. Conclusion. The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting