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Inborn Errors
Introduction
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Guidelines
Meetings

 
Introduction


The EBMT Inborn Errors of the Lympho-Hemopoetic System Working Party (IEWP) coordinates the work and interactions of centres involved in the application of BMT to treat congenital disorders of the Lympho-Hemopoetic system. In particular, these disorders include a wide spectrum of comparatively rare and heterogenous immunodeficiency disorders. These have a number of challenging aspects in common: their recognition and correct identification may be difficult, requiring special clinical experience and laboratory expertise. Patients are usually infants at high risk of rapidly developing life-threatening complications, particularly from infections. Their management requires a well-coordinated and structured approach. The prognosis can be extremely poor unless haemopoietic stem cell transplantation, which can be curative in the majority of these disorders, is performed in time.


The main intention of this Working Party is to facilitate and to intensify cooperation of transplant centres caring for patients with these rare disorders. This effort includes:

  • The use of common protocols for conditioning and performing BMT which are adjusted to specific aspects of the underlying disorders as well as to the specific donor constellations, which are commonly HLA-non-identical
  • The use of a common registry to document results of treatment
  • The analysis of treatment outcomes within classified disorders

Meetings

Members of the WP meet at least twice yearly to discuss work in progress, decide on common protocol and guidelines for treatment, formulate prospective and retrospective studies, and exchange experience.

For details of the next IEWP Meeting, please consult the Meetings section.

Subcommittees

There are a number of subcommittees of the WP that have been established to deal with aspects of particular diseases. These disorders include:

  • Osteopetrosis
  • Chronic Septic Granulomatosis
  • Metabolic Disease

Registry

The WP has a long-standing and well functioning common registry, located at Hôpital Necker in Paris, run by Prof. Alain Fischer and Dr. Paul Landais

 


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