Inborn Errors Working Party Thymic Disorders Workshop
November 10th 2014 in London, UKWhilst replacement of genetically defective haematopoietic stem cell transplantation is curative for many primary immunodeficiencies, the role of the thymus is often overlooked. However, rare disorders of thymic development, including complete DiGeorge syndrome, or FOXN1 severe combined immunodeficiency are poorly corrected by stem cell replacement, as the fundamental problem lies with thymic absence, rather than a host stem cell defect. Furthermore, thymic damage secondary to graft versus host disease also leads to poor lymphocyte immune reconstitution, with susceptibility to infection and autoimmunity.
To discuss these issues, in the context of transplantation of thymic tissue, the first Thymic Disorders workshop was held on a crisp November day in London, co-organised by Georg Hollander and Graham Davies, under the auspice of IEWP. Over 40 people gathered at Goodenough College, to discuss thymic development, developmental pathways of functional cortical and medullary thymic epithelium and targeting of T lymphocyte intra-thymic development, before focusing on primary thymic defects, effect of GvHD on thymic function, and then considering corrective strategies including engineering the thymic microenvironment, and the use of natural scaffolds to encourage thymic tissue regeneration. Finally, clinical aspects of treatment were discussed including generation of human T-lymphoid progenitors in a feeder-cell-free culture system, and human thymic transplantation. The cosy atmosphere was conducive to frank, in-depth discussion, and a further meeting is planned.
Acute Leukemia Working Party educational meeting
Unrelated Donor Hematopoietic Cell Transplantation for Acute Leukemia
November 28-29 2014 in Paris, FranceThe local host of the 7th Symposium of the Acute Leukemia Working Party was Prof Mohamad Mohty, EBMT President. The educational meeting was dedicated this year to the field of “Unrelated Donor Hematopoietic Cell Transplantation for Acute Leukemia".
The meeting was structured in three sessions. The first was devoted to donor availability; the second to impact of conditioning and the third to indications for unrelated allogeneic stem cell transplantation. The key note guest of the meeting was Prof David Klazman from Paris who gave a cutting edge superb scientific talk on induction of tolerance as therapeutic tool for various autoimmune disorders and implications for prevention and treatment of graft versus host disease (GVHD). The lecture which was followed by heavy discussion will hopefully serve as the initial basis for potential such study.
Prof Katherina Fleischhauer updated us in a seminal talk updated us on basic concepts, functional HLA matching and future directions in the field of HLA and T cells research with implications for allocating the optimal donor for unrelated transplants. Prof Fleischhauer addressed hot topics like HLA-DPBI, central memory T cells for GVL, Genomic loss of mismatched human leukocyte antigen and leukemia immune escape from haploidentical graft-versus-leukemia and more.
Prof Cornelissen discussed indication for unrelated transplants for AML on the basis of the HOVON/SAKK studies and Prof Goldstone discussed indication for unrelated transplants in ALL based on the MRC studies.
Other Faculty were: Prof Holowiecki; Ibrahim Yakub Agha and several ALWP subcommittee leaders: Savani Bipin, Baron Frederic, Giebel Sebastian and Gorin Claude. Dr. Ruggeri Annalisa discussed non T-depleted Haplo (replacing Prof Ciceri who could not attend the meeting).
We would like to thank everyone who contributed to the success of this educational event, especially the speakers and session chairs, the ALWP office and mainly Ms. Emmanuelle Polge, the supporting Pharma, the local organizers and the participants.