Hamostaseologie 2019; 39(01): 076-086
DOI: 10.1055/s-0038-1654720
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Lemierre Syndrome: Clinical Update and Protocol for a Systematic Review and Individual Patient Data Meta-analysis

Clara Sacco
1  Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
,
Federica Zane
1  Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
,
Serena Granziera
2  Department of Medicine (DIMED), Geriatric Clinic, University of Padua, Padua, Italy
3  Department of Physical and Rehabilitation Medicine, “Villa Salus” Hospital, Mestre, Italy
,
Karin Holm
4  Division of Infection Medicine, Department of Clinical Sciences, Lund University, Lund, Sweden
,
Dina Creemers-Schild
5  Department of Internal Medicine, Langeland Hospital, Zoetermeer, The Netherlands
,
Michel-André Hotz
6  Department of ENT, Head and Neck Surgery, Inselspital (University Hospital of Bern), University of Bern, Bern, Switzerland
,
Elena Turpini
7  Department of Radiology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
,
Adele Valentini
7  Department of Radiology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
,
Christian Righini
8  Department of Otolaryngology–Head and Neck Surgery, Grenoble University Hospital, Grenoble, France
,
Petros D. Karkos
9  Department of Otolaryngology–Head and Neck Surgery, AHEPA Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
,
Peter Verhamme
10  Department of Vascular Medicine and Haemostasis, University Hospitals Leuven, Leuven, Belgium
,
Marcello Di Nisio
11  Department of Medicine and Ageing Sciences, University G. D'Annunzio of Chieti-Pescara, Chieti, Italy
,
Stavros Konstantinides
12  Center for Thrombosis and Haemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Germany
13  Department of Cardiology, Democritus University of Thrace, Alexandroupolis, Greece
,
Alessandro Pecci
1  Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
,
Stefano Barco
12  Center for Thrombosis and Haemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Germany
,
on behalf of the Lemierre Study Group › Author Affiliations
Further Information

Publication History

14 August 2017

09 March 2018

Publication Date:
02 August 2018 (eFirst)

Abstract

Lemierre syndrome usually affects otherwise healthy adolescents or young adults and occurs at an overall rate of 1 to 10 cases per million person-years with an estimated fatality rate of 4 to 9%. Diagnostic criteria remain debated and include acute neck/head bacterial infection (often tonsillitis caused by anaerobes at high potential for sepsis and vascular invasion, notably Fusobacterium necrophorum) complicated by local vein thrombosis, usually involving the internal jugular vein, and systemic septic embolism. Medical treatment is based on antibiotic therapy with anaerobic coverage, anticoagulant drugs and supportive care in case of sepsis. Surgical procedures can be required, including drainage of the abscesses, tissue debridement and jugular vein ligation. Evidence for clinical management is extremely poor in the absence of any adequately sized study with clinical outcomes. In this article, we illustrate two cases of Lemierre syndrome not caused by Fusobacterium necrophorum and provide a clinically oriented discussion on the main issues on epidemiology, pathophysiology and management strategies of this disorder. Finally, we summarize the study protocol of a proposed systematic review and individual patient data meta-analysis of the literature. Our ongoing work aims to investigate the risk of new thromboembolic events, major bleeding or death in patients diagnosed with Lemierre syndrome, and to better elucidate the role of anticoagulant therapy in this setting. This effort represents the starting point for an evidence-based treatment of Lemierre syndrome built on multinational interdisciplinary collaborative studies.

Zusammenfassung

Das Lemierre-Syndrom betrifft normalerweise gesunde Jugendliche oder junge Erwachsene und tritt mit einer Gesamttodesrate von 1 bis 10 Fällen pro Million Personenjahre mit einer geschätzten Sterblichkeitsrate von 4 bis 9% auf. Diagnostische Kriterien bleiben umstritten und umfassen akute Kopf-Hals-Bakterieninfektionen (häufig Tonsillitis durch Anaerobier mit hohem Sepsis-und Gefäßinvasionspotenzial, insbesondere Fusobacterium necrophorum), kompliziert durch lokale Venenthrombose, die gewöhnlich die Vena jugularis interna involviert, und systemische septische Embolie. Die medizinische Behandlung basiert auf einer Antibiotikatherapie mit anaerober Abdeckung, Antikoagulanzien, und unterstützender Behandlung bei Sepsis. Chirurgische Maßnahmen können erforderlich sein, einschließlich der Drainage der Abszesse, Gewebedebridement und Jugularvenenligatur. Die Evidenz für das klinische Management ist in Ermangelung einer ausreichend bemessenen Studie mit klinischen Ergebnissen äußerst schlecht. In diesem Artikel illustrieren wir zwei Fälle von Lemierre-Syndrom, die nicht durch Fusobacterium necrophorum verursacht wurden und bieten eine klinisch orientierte Diskussion über die Hauptprobleme der Epidemiologie, Pathophysiologie und Managementstrategien dieser Erkrankung. Abschließend fassen wir das Studienprotokoll einer vorgeschlagenen systematischen Übersichtsarbeit und einer individuellen Patientendaten-Metaanalyse der Literatur zusammen. Unsere laufende Arbeit zielt darauf ab, das Risiko neuer thromboembolischer Ereignisse, schwerer Blutungen oder Todesfälle bei Patienten mit Lemierre-Syndrom zu untersuchen und die Rolle der Antikoagulanzien-Therapie in diesem Zusammenhang besser zu klären. Diese Bemühungen bilden den Ausgangspunkt für eine evidenzbasierte Behandlung des Lemierre-Syndroms, welche auf multinationalen interdisziplinären kollaborativen Studien aufbaut.

Authors' Contributions

Clara Sacco, Federica Zane, Serena Granziera, Marcello Di Nisio and Alessandro Pecci were responsible for the design of the study, collection of data, writing of the manuscript and gave final approval. Karin Holm and Petros Karkos were responsible for the writing of the manuscript, critical revision of the manuscript and gave final approval. Elena Turpini and Adele Valentini were responsible for collection of data, critical revision of the manuscript and gave final approval. Dina Creemers-Schild, Michael-André Hotz, Christian Righini, Peter Verhamme and Stavros Konstantinides critically revised the manuscript and gave final approval. Stefano Barco was responsible for the concept and design of the study, collection of data, quality assessment and data analysis, writing of the manuscript and gave final approval.


Supplementary Material