Inborn Errors Working Party (IEWP)

IEWP Newsletter December 2017

by Arjan Lankester

Annual ESID-EBMT Inborn Errors WP (IEWP) meeting and the combined IEWP and Autoimmune Diseases WP (ADWP) educational meeting in Newcastle 3rd-5th November 2017

Dear friends,

This year, the IEWP annual autumn meeting was held in Newcastle, UK. The first day of the conference (3rd November) was a special educational day organized in a joint effort by IEWP and Autoimmune Diseases WP entitled “Interface between Autoimmunity and Primary Immune Deficiency”.

The excellent and stimulating educational program (see website for details) included presentations on the activities from both WPs, PID as cause of autoimmune disease (AID), role of allo-HSCT in a AID, targeted therapies for autoimmune lympoproliferative PID, auto-HSCT in Crohn disease, systemic sclerosis, and juvenile arthritis, and immune reconstitution after auto- and allo-HSCT. The final keynote lecture was given by Mario Abinun, a longstanding member of both IEWP and ADWP, on the role of allo-HSCT for juvenile arthritis and autoinflammatory disease.

The second day of the meeting included sessions on HSCT and gene therapy (GT) in metabolic diseases and DNA repair disorders, HSCT in emerging indications and novel primary immune disorders, and experience with SCID newborn screening in Europe and North America. The day was concluded with an outstanding key note lecture given by Georg Holländer on the impact of PID and GvHD on thymic function.

On the final day, specific sessions focussed on HSCT and GT in beta-thalassemia, and multicenter experiences with novel strategies in haplo-identical transplantation. The meeting was concluded with a session dedicated to our current IEWP studies, novel study proposals including collaborative studies with our North American colleagues from PIDTC, and a contribution by our IEWP EBMT data officer.

The meeting was very well attended by around 130 participants mainly coming from Europe, but with a substantial number of colleagues from North America, Australia, Asia and Latin America. I would like to thank all participants for their excellent presentations and contributions in the discussions, and Andy Gennery and Roz Scott for being perfect hosts.

We look forward to several exciting IEWP activities in 2018. First, we will meet at the annual EBMT meeting in Lisbon (March 18-21), where we will have our dedicated IEWP sessions. In June (7-9, Verona), the EBMT Pediatric Diseases WP, Infectious Diseases WP and IEWP will organize a joint bi-annual meeting. The IEWP annual meeting will be organized in Leiden, September 28-30, 2018. During this meeting we will celebrate the 50th anniversary of the first successful SCID transplant in Europe with a special educational first day. Further details on these meetings will be communicated soon.

I look forward to meet you all during these meetings and wish you all the best for 2018.

Arjan Lankester