Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: An eight-year follow-up

Daguindau, Nicolas; Decot, Véronique; Nzietchueng, Rosine; Ferrand, Christophe; Pïcard, Capucine; Latger‐Cannard, Véronique; Gregoire, M.; Béri, Mylène; Salmon, Alexandra; Stoltz, J.F.; Bordigoni, Pierre; Bensoussan, Danièle

doi:10.1016/j.clim.2008.03.524

Cited by 12 publications

(11 citation statements)

References 33 publications

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“…Despite the lack of a functional thymus to mature lymphoid progenitors there is peripheral expansion of lymphocytes providing adequate protection against infection. However, tolerance does not develop and the risk of GvHD is high[25,26]. Thymic transplant has been recently developed for patients with complete DiGeorge anomaly.…”

Section: Introductionmentioning

confidence: 99%

Clinical experience in T cell deficient patients

Cole

Cant

2010

All Asth Clin Immun

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T cell disorders have been poorly understood until recently. Lack of knowledge of underlying molecular mechanisms together with incomplete data on long term outcome have made it difficult to assess prognosis and give the most effective treatment. Rapid progress in defining molecular defects, improved supportive care and much improved results from hematopoietic stem cell transplantation (HSCT) now mean that curative treatment is possible for many patients. However, this depends on prompt recognition, accurate diagnosis and careful treatment planning.This review will discuss recent progress in our clinical and molecular understanding of a variety of disorders including: severe combined immunodeficiency, specific T cell immunodeficiencies, signaling defects, DNA repair defects, immune-osseous dysplasias, thymic disorders and abnormalities of apoptosis.There is still much to discover in this area and some conditions which are as yet very poorly understood. However, with increased knowledge about how these disorders can present and the particular problems each group may face it is hoped that these patients can be recognized early and managed appropriately, so providing them with the best possible outcome.

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Section: Introductionmentioning

confidence: 99%

Clinical experience in T cell deficient patients

Cole

Cant

2010

All Asth Clin Immun

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“…[131][132][133][134][135][136][137] For complete DiGeorge anomaly, a review of 17 treated patients demonstrated 41% overall survival. 136 Although patients develop normal T cell numbers through clonal expansion and normal proliferative responses to PHA, a significant issue that remains unaddressed involves persistent lack of thymic function.…”

Section: Bone Marrow Transplant or Mature T-cell Infusionsmentioning

confidence: 99%

Severe Combined Immunodeficiency Disorders

Chinn

Shearer

2015

Immunology and Allergy Clinics of North America

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“…In reviewing other patients who underwent other similar transplants, we identified 9 transplants performed in 22qDS patients [16][17][18][19][20][21][22][23][24], 6 of whom were known to be alive at between 2 and 24 years post transplant. In general, these patients showed reduced T-cell numbers and no TRECs, suggesting that the T cells were generated through extra-thymic processes.…”

Section: Adoptive Transfer Of Mature T Cells (Atmtc)mentioning

confidence: 99%

Immunologic reconstitution in 22q deletion (DiGeorge) syndrome

2009

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Adoptive transfer of mature T cells (ATMTC) through bone marrow (BM) transplantation, first attempted over 20 years ago, has recently emerged as a successful therapy for complete 22q deletion syndrome (22qDS). This provides a potential option to thymic transplantation (TT) for immune reconstitution in 22qDS. Compared to thymic transplant, ATMTC is an easier procedure to accomplish and is available at more centers. However, there are differences in the nature of the T-cell reconstitution that results. Predictably, more naïve T cells and recent thymic emigrants are present in patients treated with thymus transplant. There are no significant differences in mortality between the two procedures, but the number of patients is too limited to conclude that the procedures are equally effective. Adoptive transfer should be pursued as a reasonable treatment for 22qDS patients requiring immune reconstitution when thymus transplant is not available.

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Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: An eight-year follow-up

Cited by 12 publications

References 33 publications

Clinical experience in T cell deficient patients

Clinical experience in T cell deficient patients

Severe Combined Immunodeficiency Disorders

Immunologic reconstitution in 22q deletion (DiGeorge) syndrome

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