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European initiative to improve knowledge, treatment and survival of haemophagocytic syndromes in children

European initiative to improve knowledge, treatment and survival of haemophagocytic syndromes in children

Objective

Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening inherited immune disorder of early childhood characterised by an uncontrolled immune response. Three disease causing genes have been identified in about half of the patients. Diagnosis is difficult, pathophysiology poorly understood, and treatment unsatisfactory with about 40% of the children dying from treatment failure or toxicity. All European scientists active in research and treatment of HLH have joined in a consortium to enforce collaboration, combine the various expertises, and provide access to material and data. In vitro studies and several murine models for HLH and diseases predisposing to HLH, will be used to get more insight into the pathophysiology of the diverse genetic defects. Studies will address the role of triggers and antigen-presenting cells, the influence of cytokines in the initiation and propagation of the uncontrolled immune response, the interaction of immune effector cells and soluble factors, and the mechanism of cytotoxicity in lymphocytes and natural killer cells. Data about geno-phenotype will be combined to evaluate how known and unknown genetic defects reflect on the clinical course and immunophenotype of the patients. A diagnostic algorithm, incorporating knowledge from the known defects, will be developed and optimised in all countries to facilitate earlier and reliable diagnosis, and to separate patients with unknown defects or acquired HLH. The identification of risk factors for treatment failure and toxicity will be used to design improved treatment strategies. An experimental treatment will be tested in a murine model and will be the basis for a less toxic treatment in the future. Earlier diagnosis, better insight into the pathophysiology, and development of less toxic treatments are the steps towards a higher cure rate in HLH. In a rare disease as HLH, only combined efforts on an European-wide level will be able to achieve these goals.
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Coordinator

UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF

Address

Martinistrasse 52
20251 Hamburg

Germany

Activity type

Higher or Secondary Education Establishments

EU Contribution

€ 546 987

Administrative Contact

Kai Lehmberg (Dr.)

Participants (8)

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UNIVERSITA DEGLI STUDI DI BRESCIA

Italy

EU Contribution

€ 346 200

FORSCHUNGSZENTRUM BORSTEL

Germany

EU Contribution

€ 548 213

UNIVERSITAETSKLINIKUM FREIBURG

Germany

EU Contribution

€ 486 920

THE CHANCELLOR MASTERS AND SCHOLARS OF THE UNIVERSITY OF CAMBRIDGE

United Kingdom

EU Contribution

€ 72 170

KAROLINSKA INSTITUTET

Sweden

EU Contribution

€ 300 720

INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE

France

EU Contribution

€ 414 330

AZIENDA OSPEDALIERO-UNIVERSITARIA ANNA MEYER

Italy

EU Contribution

€ 168 852

ISTITUTO GIANNINA GASLINI

Italy

EU Contribution

€ 110 550

Project information

Grant agreement ID: 201461

Status

Closed project

  • Start date

    1 June 2008

  • End date

    31 May 2011

Funded under:

FP7-HEALTH

  • Overall budget:

    € 3 917 550,34

  • EU contribution

    € 2 994 942

Coordinated by:

UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF

Germany